Rare case of leiomyoma and endometriosis in Mayer–Rokitansky–Kuster–Hauser Syndrome

We report a case of leiomyoma with endometriosis that developed from a hypoplastic uterus in a patient with Mayer–Rokitansky–Kuster–Hauser syndrome. A 32-year-old patient with primary amenorrhea and primary infertility presented with an abdominal mass and abdominal pain. She is phenotypically female, has well developed secondary sexual characteristics, and has normal female external genitalia with blind vaginal pouch. Transabdominal ultrasonography showed a homogenous left adnexal mass of 8 cm × 10 cm originating from the left ovary, which was not seen separately. Uterus rudimentary with normal right ovary. A complex mass of the left adnexa was demonstrated by computed tomography scan, with the possibility of torsion. Exploratory laparotomy showed torsion of the right endometriotic (chocolate) cyst and rudimentary uterus with a huge fibroid of size 8 cm × 10 cm with endometrial deposits all over the peritoneum. The left ovary was normal. Removal of leiomyoma with rudimentary uterus with absent cervix was done. Right oophorectomy with cystectomy was done with preservation of the left ovary. The histologic diagnosis was leiomyoma with endometriosis, with an endometriotic cyst arising from the right adnexa.