RESUMO
The urachus is a vestigial structure located between the dome of the bladder and the umbilicus, which results from the involution of the allantoic duct and the cloaca. Persistence of an embryonic urachal remnant can cause various problems during childhood and young adulthood. Urachal leiomyoma is a rare entity with very few cases being reported in literature. It can be misdiagnosed and confused with a wide spectrum of intra-abdominal or pelvic disorders. We hereby report a case of angiomatous leiomyoma originating from the urachal remnant in a 45-year-old lady, masquerading as extraluminal gastrointestinal stromal tumor. Understanding the embryological basis of these urachal disorders and their imaging features coupled with histopathological examination is crucial for the correct diagnosis and management. Pathological diagnosis is required to optimize the surgical approach and preclude unnecessary radical surgery.