RESUMO
In this case report, a Mayer-Rokitansky-Kuster-Hauser syndrome with pelvic ectopic kidney and a perirenal cyst with endometrial tissue inside is demonstrated. A 17 year old patient admitted with primary amenorrhea. Pubertal stages were completed. In pelvic ultrasonography; uterus could not be detected, a 6x11 cm sized cystic lesion was seen on the right adnexal area. A centrally located 5.5x9 cm sized ectopic pelvic kidney was detected. Hormones and tumor markers were normal. Laparoscopy was planned. In the laparoscopic observation, uterus and both tubes could not be detected, ovaries were normal. There was a 6x7 cm sized cyst located in the retroperitoneal area, the origin of the cyst could not be identified. Laparatomy was considered, retroperitoneal space was entered, an 8x11 cm sized smooth contoured perirenal cyst adjacent to the pelvic kidney was detected. Cyst was extirpated. The pathology result was reported to include endometrial tissue and hemorrhage inside