Herlyn-Werner-Wunderlich Syndrome Complicated with Vesicovaginal Fistula: A Rare Case Report.

Herlyn-Werner-Wunderlich (HWW) syndrome is characterized by obstructed hemivagina and ipsilateral renal anomaly, a rare congenital anomaly of the genitourinary tract, resulting from malformations of the renal tract associated with Müllerian duct anomalies. The initial symptoms of HWW frequently present after menarche and may be nonspecific, leading to a delayed diagnosis. We presented a 19-year-old female with 3-year hematuria and abdominal pain. The final diagnosis of HWW syndrome with a rare vesicovaginal fistula was made. The treatment of HWW syndrome typically involves surgical intervention. The primary treatment is resection or removal of the obstructed vaginal septum. The patient underwent excision of vaginal septum and vaginal reconstruction via hysteroscopy, as well as repair of the vesicovaginal fistula. The patient improved well after surgery and fully recovered without sequelae after 3 months. In addition, unilateral renal agenesis is one of congenital abnormalities of the kidney and urinary tract, which are the most frequent cause of chronic kidney disease (CKD) in children. This report describes a patient of HWW syndrome with rarely combined vesicovaginal fistula, and highlights the importance of early recognition and management to prevent associated complications.

True uterus didelphys in she-camel: a case report and review of literature.

Background: Uterus didelphys is a rare congenital anomaly of the female reproductive tract characterized by a divided uterine cervix and body. It occurs due to abnormal development of the paramesonephric (Müllerian) duct. Different forms of uterus didelphys have been reported in several animal species, including bovine, equine, ewe, goat, swine, and bitch. However, there is no previous report that has documented a completely divided female genital tract in she-camel. Moreover, there is a lack of literature regarding this anomaly in animals. Therefore, the present study reports, for the first time, a rare case of a completely divided female genital tract in a she-camel. In addition, the existing relevant literature on uterus didelphys in different animal species is reviewed. Case presentation: A female reproductive tract of she-camel, approximately 10 years old, with a history of previous successful pregnancy, was brought to the anatomy department following the slaughtering of the animal. Initial examination revealed a normal reproductive tract consisting of two ovaries, two fallopian tubes, a uterus, and a vagina. A closer examination revealed a completely divided vagina, with an external os opened into each part of the vagina, as well as a divided uterine body and cervix. Intrauterine infusion of saline through one external os confirmed complete separation of uterine body and cervix. Conclusion: To the authors' knowledge, this is the first reported case of a completely divided female genital tract in a she-camel. This review summarizes the previous reports about uterus didelphys in farm animals.

A case of Herlyn-Werner-Wunderlich syndrome with exacerbation of hematometra after adnexectomy.

A 27-year-old nulliparous woman presented with a feeling of fullness in the lower abdomen and abdominal pain. A left ovarian tumor, uterus didelphys, left renal agenesis, and left vaginal atresia were observed on imaging. The ovarian tumor was presumed to have caused the abdominal pain, and an abdominal left adnexectomy was performed. After 3 months, she reported severe lower abdominal pain during menstruation. Transvaginal ultrasonography revealed uterine enlargement. After 17 days, the patient presented with abdominal pain and fever. She was diagnosed with peritonitis due to infection and left uterine hematometra. Because she did not improve with antibiotic treatment, left laparoscopic hysterectomy was performed. Subsequently, she did not experience the lower abdominal pain. Appropriate diagnosis and treatment based on the morphology of the reproductive tract and symptoms must be considered in patients with Herlyn-Werner-Wunderlich syndrome. Treatment must permit the outflow of menstrual blood.

Undiagnosed Uterine Didelphys in a Multiparous Somali Woman with Two Previous Cesarean Sections.

Background: Uterine didelphys (UD) develops from failure of fusion of the paired Müllerian ducts, resulting in two noncommunicating uteri. We present a 31-year-old pregnant woman whose UD anomaly had not been detected during two previous cesarean sections and her presentation to a health-care clinic for her fifth pregnancy. Case Presentation: She was referred to our obstetrics clinic due to suspicion of abdominal pregnancy and a complaint of severe lower abdominal pain. On ultrasonography, UD was detected with two adjacent uteri, one of which was empty and the other with a fetus of approximately 1100 g at 28 weeks and 1 day of gestational age. Magnetic resonance imaging confirmed the presence of UD. Due to severe lower abdominal pain of the patient and severe oligohydramnios of the fetus, emergency cesarean section was performed, and a 980 g male baby was delivered. Conclusion: This case exemplifies how difficult life is for women living in an underdeveloped and resource-limited country like Somalia.

An Incidental Diagnosis of Herlyn-Werner-Wunderlich Syndrome in a Young Female: A Case Report of a Rare Entity.

Obstructed hemivagina and ipsilateral renal anomaly (OHVIRA) syndrome, a rare genetic condition of the urogenital system, is characterized by distinctive features such as ipsilateral renal agenesis, obstructed hemivagina, and uterus didelphys. It is also referred to as Herlyn-Werner-Wunderlich (HWW) syndrome. Its clinical manifestations include dysmenorrhea, consistent abdominal pain, and infrequent periods. It is typically diagnosed after menarche. We report a case of a 20-year-old female who was admitted to the casualty ward following a road accident. She was Incidentally found to have uterine didelphys with hemorrhagic cystic lesion and left renal agenesis on ultrasonography (USG). She also had gallbladder stones, along with the findings mentioned above. Clinicians should exclude HWW syndrome in cases where uterine didelphys and unilateral renal agenesis coexist. Prompt identification and treatment of the condition can help avoid potential untoward pregnancy-related issues in the future.

Herlyn–Werner–Wünderlich syndrome: A case series / Síndrome de Herlyn-Werner-Wünderlich: una serie de casos

Introduction: Herlyn–Werner–Wünderlich syndrome is a uterine malformation characterized by uterus didelphys, obstructed hemivagina and ipsilateral renal agenesis. Clinical findings: The manifestation of the disease is widely diverse; it is usually diagnosed after menarche, with dysmenorrhea and abnormal uterine bleeding; it is also associated with infertility. Main diagnosis: Four clinical cases, their diagnosis are reported here. Therapeutic interventions and results: The treatment and results of these four patients are described here. Conclusion: When studying uterine malformation it is important to consider this rare disease to avoid possible complications and giving the patient a correct diagnose and treatment. The hysteroscopy resection of the longitudinal vaginal septum in those symptomatic patients with hematocolpos should be considered as a good option for treatment.(AU)

Introducción: El síndrome de Herlyn-Werner-Wünderlich es una malformación uterina que asocia útero didelfo, hemivagina obstruida total o parcialmente y agenesia renal ipsilateral. Hallazgos clínicos: La clínica que presenta este síndrome es muy diversa; se suele diagnosticar después de la menarquia cursando con dismenorrea y sangrado uterino anómalo; así mismo se asocia a infertilidad. Diagnósticos principales: Se presentan a continuación 4 casos clínicos, su diagnóstico y tratamiento mediante diversas técnicas. Intervenciones terapéuticas y resultados: Se describen en este manuscrito los tratamientos aplicados a estas pacientes y sus resultados. Conclusión: Ante el hallazgo de una malformación uterina es importante tener en cuenta esta entidad infrecuente, para evitar posibles complicaciones y proporcionar a la paciente un diagnóstico y tratamiento correctos. La resección histeroscópica del tabique vaginal longitudinal en aquellas pacientes sintomáticas con hematocolpos debe ser considerada como una buena opción de tratamiento.(AU)

Laparoscopic Uterine Preservation and Vaginal Reconstruction in a 13-Year-Old Girl with Uterus Didelphys, Bilateral Vesicovaginal Fistulas, and Transverse Vaginal Septum.

BACKGROUND: Complex female genital tract malformations account for 1.2% of all female genitourinary malformations. Although exceedingly rare, they can cause severe gynecologic symptoms in young women and lead to fertility problems. CASE: We present the case of a 13-year-old girl with primary amenorrhea referred for cyclic abdominal lower pain and menouria. Detailed diagnostics revealed uterus didelphys, transverse vaginal septum, and bilateral vesicovaginal fistulas. Laparoscopic left hemi-hysterectomy and salpingectomy were performed. The vesicovaginal fistula on the right side was excised, and the proximal vagina was anastomosed with the distal dimple. Since the operation, the patient has been pain-free and menstruating regularly from the right uterus. SUMMARY AND CONCLUSION: Preservation of the uterus should be considered in any case of complex female genital tract malformation and, as successful laparoscopic treatment advocates, a minimally invasive approach is feasible.

"Can't Touch This!": Laparoscopic management of an obstructed uterus didelphys before and after treatment for pelvic inflammatory disease.

OBJECTIVE: To describe the laparoscopic management of an obstructed uterus didelphys before and after treatment for pelvic inflammatory disease. To compare the appearance of pelvic organs during active infection with their appearance after washout and appropriate antibiotic treatment, emphasizing the importance of knowing when to abort a procedure. DESIGN: Video demonstration of surgical and medical management considerations during a complex pelvic surgery. Visualization of tissue healing that occurs with appropriate antibiotic treatment. SETTING: Academic Center. PATIENT: A patient who presents for definitive surgical management of a uterus didelphys with an obstruction at her right hemicervix. Her presentation is complicated by a tubo-ovarian abscess. INTERVENTION: A uterus didelphys is classically defined as two hemiuteri with duplicated cervices with or without a longitudinal vaginal septum. Uterus didelphys may have an obstruction and/or communication between the two uterine horns, in which case patients may present with complications such as cyclic pelvic pain from hematometra or genital tract infection. This is a case report of a 14-year-old G0 who presented to the emergency department with two weeks of vaginal bleeding, severe diffuse abdominal pain, and malodorous vaginal discharge. Transabdominal ultrasound and a magnetic resonance imaging of the pelvis established a new diagnosis of a uterus didelphys with an obstruction at her right hemicervix and a fistulous tract connecting her right and left hemiuteri at the level of the internal cervical os. She was also found to have a 3 cm left ovarian cyst and a new finding of congenital absence of her right kidney. Patient was administered ceftriaxone, doxycycline, and metronidazole antibiotics as treatment of presumed pelvic inflammatory disease but experienced minimal improvement after 24 hours. The decision was made to proceed with surgical intervention. A survey of the pelvis revealed significant inflammation, friable peritoneum, and endometriosis. The uterine horns in didelphic configurations were visualized. The fimbriae at the left fallopian tube were notably splayed out, swollen, and inflamed. There was a notable large mass in the location where the ovarian cyst had been previously described on imaging. A large amount of purulent material was expressed when compressed, consistent with a tubo-ovarian abscess. The infection likely originated from the menstrual blood collection at the right obstructed cervix that ascended through the communication between the right and left hemiuteri. The pelvis was irrigated thoroughly. At this point, the decision was made to stop the procedure, pursue antibiotic treatment, and resolve the active infection before correcting her complex müllerian anomaly. Patient continued on her antibiotic course, which included piperacillin-tazobactam, while hospitalized, followed by a five-day course of amoxicillin-clavulanate. She was also placed on medroxyprogesterone acetate for menstrual suppression. MAIN OUTCOME MEASURE: Advantage of allowing time for antibiotic treatment and tissue healing before repair of a complex müllerian anomaly. RESULT: With antibiotic treatment, she recovered well postoperatively with resolution of her pain. Three months later, she returned to the operating room for definitive surgical management of her obstructed uterine didelphys. On laparoscopy, there was a significant improvement in tissue quality. Most notably, the fimbriae of the left fallopian tube were no longer inflamed. We proceeded with the planned correction of the complex müllerian anomaly. After resection of the right uterine horn, the fistula tract was identified and also resected. The defect in the right hemicervix was closed over, reinforcing the medial side of the left hemicervix. She had an uncomplicated postoperative recovery, and menses resumed without pain. CONCLUSIONS: The presented case provides unique insight into the tissue healing that occurs before and after antibiotic treatment. Knowing when to stop, especially in the setting of an active infection, is extremely important for performing a procedure safely, minimizing harm, and allowing for robust tissue repair. It is also important to optimize modifiable preoperative factors before correcting a complex müllerian anomaly. Assessing and reassessing the situation during a complex pelvic surgery is essential, especially in the setting of a complex müllerian anomaly where the preoperative examination and imaging may not be definitive.

Presentation and Management of Uterine Didelphys with Unilateral Cervicovaginal Agenesis/Dysgenesis (CVAD): A Multicenter Case Series.

INTRODUCTION: There are several well-described presentations of uterine didelphys (UD): UD without vaginal septum, UD with non-obstructed longitudinal vaginal septum, or UD with duplicated vaginas and an obstructed hemivagina on one side with ipsilateral renal anomaly. STUDY OBJECTIVE: To describe another variant of UD and compare the presentation and management across different institutions METHODS: This was a retrospective case series approved by the NASPAG Fellows Research Consortium. Participating institutions obtained IRB approval. Inclusion criteria included a diagnosis of UD and unilateral cervicovaginal agenesis/dysgenesis (CVAD). Descriptive statistics were used. RESULTS: Five patients met the inclusion criteria, with ages ranging from 13 to 27 years. Presenting symptoms included dysmenorrhea (80%), irregular bleeding (40%), acute onset left lower quadrant pain (20%), and abdominal mass (20%). Three patients had additional known abnormalities, including solitary kidney and solitary adrenal gland. All patients underwent pelvic magnetic resonance imaging. Two cases were only suspicious for unilateral CVAD on imaging and required pathology review postoperatively to confirm diagnosis. Two cases required a 2-staged approach with an initial diagnostic surgery followed by a second definitive procedure. Three patients were noted to have endometriosis intraoperatively. Postoperative follow-up ranged from 2 months to 2 years, with 1 patient reporting chronic pelvic pain. CONCLUSION: Diagnosis on the basis of pelvic imaging can be difficult, as this unique variant may mimic classic obstructed hemivagina with ipsilateral renal anomaly. In patients with UD with unilateral CVAD, standard management is removal of the obstructed uterine horn. This multicenter series stresses awareness about the clinical presentation, distinguishes cases of cervical agenesis from dysgenesis, and reviews approaches to management.

Hemihysterectomy in a patient with uterus didelphys, vaginal septum and ipsilateral renal agenesis: A case report and literature review.

Uterus didelphys is a rare Müllerian anomaly, often diagnosed during menarche or in women with a personal history of infertility and/or recurrent pregnancy loss. Its association with other genitourinary anomalies is frequent and may determine the existence of established syndromes. This case report refers to a 13-year-old female patient diagnosed with OHVIRA syndrome (Obstructed Hemivagina with Ipsilateral Renal Agenesis), a condition wherein the presence of a didelphic uterus is associated with hemivagina obstruction and ipsilateral renal agenesis. The patient presented with cyclic pelvic pain, related to the presence of hematocolpos and hematometra, which persisted despite several surgical approaches, including vaginal septum excision and correction of cervical stenosis. The recurrence of the condition indicated exploratory laparotomy, revealing two hemi-uteri and two uterine cervixes, with hematometra on the right. A subtotal hemihysterectomy was performed on the right. Post-procedure, the patient developed with regular menstrual cycles and improvement of pelvic pain complaints. Given the limited prevalence and low index of suspicion, the potential requirement for surgical intervention and its potential impact on reproductive future, diagnosing and treating OHVIRA syndrome and other Müllerian anomalies poses notable challenges in clinical practice. Hence, sharing different therapeutic approaches of a rare diagnosis with the scientific community is of paramount importance to aid in early diagnosis and effective management of similar clinical cases.

Endometrial Cancer in a Patient With Didelphys Uterus and a History of Renal Cancer: A Case Report and Literature Review.

Müllerian duct anomalies (MDAs) concurrent with endometrial cancer are exceptionally rare, with only a few documented cases. Here, we present a case of endometrial cancer in both horns of a didelphys uterus in a 54-year-old woman with a history of renal cancer, who underwent left radical nephrectomy and left salpingo-oophorectomy. The patient sought medical evaluation due to postmenopausal vaginal bleeding. Hysteroscopy with dilation and curettage revealed the presence of two cervixes and two endometrial cavities, with pathology results indicating endometrioid adenocarcinoma (G1). Preoperative MRI staging confirmed the diagnosis of a double cervix and uterus. Subsequently, an open abdominal hysterectomy and a right salpingo-oophorectomy were performed, revealing a didelphys uterus (International Federation of Gynaecology and Obstetrics 2018, stage IA). This manuscript aims to explore the potential correlation between renal and endometrial malignancies in the presence of MDAs.

MRI Findings in a Rare Case of Left-Sided Obstructed Hemivagina and Ipsilateral Renal Anomaly (OHVIRA) Syndrome in a 21-Year-Old Female.

Obstructed hemivagina and ipsilateral renal anomaly (OHVIRA) is a complex Mullerian and Wolffian duct anomaly, which is difficult to diagnose before puberty. We present a rare case of a congenital syndrome known as OHVIRA in a 21-year-old female who came with complaints of intermittent type of lower abdominal pain, dysmenorrhea, and oligomenorrhea with frequent visits to different hospitals without any radiological investigations done. Early magnetic resonance imaging (MRI) investigations helped her in diagnosing and managing this syndrome.

Uterocolon Fistula Formation in 50 Year Old Patient with History of 16 Years Intrauterine Device Use: A Case Report.

Introduction: Uterocolon fistula is one of the complications of intrauterine device (IUD) insertion. Not only may IUD materials cause perforation, but some other risk factors may contribute to its development including uterine abnormalities, thus IUD is contraindicated in patients with anatomical anomaly. Case: P3A1 woman, 50 years old with a history of IUD use for 16 years presented with complaints of fecal discharge from the vagina 8 months ago which worsened after IUD extraction. Physical examination revealed no abdominal tenderness. Speculum examination found feces in the cervical canal. CT scan examination showed multiple uterocolon fistulas and uterine didelphys. Diagnostic laparoscopy and hysteroscopy were carried out and found a recto-uterine fistula, then the patient was scheduled for colostomy and reanastomosis with the stapler method. Conclusion: Diagnosis was very difficult to establish despite proper imaging modalities. The use of direct visual diagnostics (hysteroscopy and laparoscopy) can be a good alternative for the diagnosis of uterocolon fistula. To the best of our knowledge, this is the first case report on recto-uterine fistula in a patient with long-term use of IUD and uterine didelphys.

Uterine Leiomyoma in the Context of Uterine Didelphys: A Case Report.

Uterine leiomyomas are one of the most common reproductive pathologies in born females. The majority of women within reproductive age will develop a leiomyoma, most of which will be asymptomatic. Though there has been extensive research regarding this pathology alone, there is more to be learned about leiomyomas that affect women with other comorbidities. This case study reviews the medical and surgical management of a woman born with two uteri, medically termed congenital uterus didelphys. Within her reproductive years, she develops symptomatic leiomyomas in each of her uteri and seeks surgical management. This case study aims to widen the scientific knowledge surrounding these subsets of women with a common diagnosis superimposed on an extremely rare diagnosis.

Herlyn-Werner-Wunderlich syndrome: A fertility-sparing approach to a rare mullerian anomaly.

The Herlyn-Werner-Wunderlich syndrome (HWWS) is characterized by the triad of uterus didelphys, obstructed hemivagina, and renal agenesis. The typical clinical presentation involves chronic pelvic pain, dysmenorrhea, and palpable abdominal mass, related to hematocolpos/hematometra. It is a rare disease, with a challenging clinical and radiological diagnosis. Surgery is the definitive treatment. Complications such as endometriosis, infertility and chronic pelvic pain occur more frequently and severely when diagnosis and treatment are delayed. This is a case report of a twelve-year-old patient admitted to the Gynecology Department of the Federal University of Rio de Janeiro's General Hospital (HUCFF/UFRJ), in March 2021, with progressive symptoms of dysmenorrhea and abdominal distention due to palpable abdominal mass. She had a previous history of congenital solitary kidney. Magnetic Resonance Imaging (MRI) showed a double uterus with hematometra and hematocolpos on the left side, pelvic endometriosis and left renal agenesis. Conservative clinical treatment with inhibition of the hypothalamic-pituitary-ovarian (H-P-O) axis was initiated while a definitive surgical approach was being defined. In June 2022, the patient underwent left hemi-hysterectomy and salpingectomy, achieving full remission of symptoms. Given the rarity of this syndrome and its potential complications, our report aims to familiarize clinicians with it, mostly those who work with children and adolescents, so that more patients have access to early diagnosis and adequate treatment. Consequently, future fertility can be effectively preserved.

Undiagnosed uterus didelphys with unicavitary twin gestation in a district hospital in Ghana: a case report.

Uterus didelphys is a congenital anomaly of the female reproductive tract which arises from the abnormal fusion of the Mullerian ducts. We present, the first case to the best of our knowledge, of uterus didelphys with a unicavitary twin gestation to be documented in Ghana, a low-middle income country. A 24-year-old woman, gravida 3, para 0+2 miscarriages, was seen and admitted to our maternity ward due to elevated blood pressure with ++ proteinuria at 36 weeks of gestation. She attended an antenatal clinic regularly during the pregnancy but was mainly seen by midwives. Apart from multiple pregnancy, two 2D ultrasound examinations (one at 25 weeks gestation and another during admission) did not reveal any uterine malformations. At 37 weeks+2 days, she underwent emergency cesarean section on account of pre-eclampsia and a twin pregnancy with the leading twin in breech presentation. After delivering both babies and the placenta, the uterus was exteriorized and inspected, during which a non-gravid bulky left uterus was first found. Each uterus had a normal ovary and fallopian tube on its lateral end. Further postoperative examination revealed a normal-looking vulva, two vaginas, and two cervices. Both babies weighed 1.9 kg, each below the fifth percentile of weight for age. The elevated blood settled postoperatively and the postoperative period was uneventful. The patient and twins were found in a stable condition on review two weeks after delivery and the twins were healthy at 5 years. Despite being a rare presentation, we wish to create awareness among health workers in rural and low-resource settings of such cases and highlight the need to improve prenatal diagnostic capabilities, as this is key to determining the mode of delivery and achieving favorable maternal and fetal outcomes.

Herlyn-Werner-Wunderlich syndrome also known as obstructed hemivagina and ipsilateral renal anomaly: A case report and a comprehensive review of literature.

Herlyn-Werner-Wunderlich syndrome, also known as obstructed hemivagina and ipsilateral renal anomaly (OHVIRA), is a Müllerian duct anomaly. It is a rare clinical condition consisting of a duplicated uterus with an oblique vaginal septum that causes partial genital tract outflow obstruction. A urinary tract anomaly, most commonly renal agenesis, is usually present on the obstructed side. The diagnosis of genital tract outflow obstruction is often delayed due to the normal functioning of the unaffected side. The most frequent complications are dysmenorrhea, chronic pelvic pain, infection, infertility and endometriosis. This report describes a 17-year-old G0P0 patient with a history of severe dysmenorrhea and left-sided renal agenesis, who was admitted for complaints of foul vaginal discharge over the past 3 months that was unsuccessfully treated with antibiotics. Transrectal ultrasound revealed the presence of 2 separate hemicavities on transverse and longitudinal views. A cystic lesion with ground-glass opacities was detected between the bladder and a normal-appearing cervix, which was determined to be hematocolpos. The diagnosis of OHVIRA was made. This case highlights the importance of excluding a Müllerian anomaly in the presence of renal system abnormalities. Being aware of the type of anomalies, combinations and variants is crucial to determine the diagnosis and the best surgical approach. Ultrasound was an invaluable imaging exam to determine the type of anomaly and its complexity. Awareness of this syndrome and its variants will prevent misdiagnosis and will help to define the appropriate treatment for these patients.

Dicavitary twin pregnancy in patient with bicornuate bicollis uterine anomaly.

Key Clinical Message: Twin pregnancies in uterine didelphys and uterus bicornuate bicollis represent dicavitary twin pregnancies that can be managed using similar principles. Consideration must be given to delivery planning including mode of delivery and uterine incision. Abstract: Dicavitary twin pregnancies present unique challenges for obstetric management. This case demonstrates an approach to management of a bicornuate bicollis twin pregnancy and provides a contemporary review of the literature on dicavitary twin pregnancies.

Obstructed Hemivagina and Ipsilateral Renal Agenesis Syndrome in a Neonate.

Obstructed hemivagina with an ipsilateral renal anomaly (OHVIRA) syndrome is a congenital malformation that presents as a uterine didelphys with an obstructed hemivagina and an associated ipsilateral renal aberration. The clinical symptoms usually manifest after menarche. Unlike the typical presentation in adolescence, this case report features a neonatal presentation of OHVIRA syndrome with an unusual renal association. A female twin delivered at 35 weeks of gestation was transferred to our institution after birth from an outside hospital due to respiratory distress and for evaluation of the left multicystic dysplastic kidney identified on prenatal ultrasound. Physical examination and lab results, including a complete blood count, and a basic metabolic panel, including blood urea and serum creatinine, were within the normal range for age. Abdominal and pelvic ultrasound showed multicystic dysplastic left pelvic kidney, congenital hepatic cyst measuring 6 mm, uterine didelphys with duplication of the vaginal canal, and obstructed left hemivagina corresponding to the OHVIRA syndrome. Further testing revealed a normal chromosomal microarray, small patent foramen ovale on the echocardiogram, no vertebral or rib anomalies on the spinal x-ray, normal hearing test, and mild optic cupping on the ophthalmological evaluation. The pediatric surgeon and urologist recommended an outpatient follow-up and elective surgery in the future. This is a unique case presenting in the neonatal period with an unusual association. Timely intervention can help prevent obstetric complications.