Ectopic Pelvic Kidney with Lithiasis Presenting as Acute Abdomen in Mayer-Rokitansky Syndrome: Report of a Case

The ectopic pelvic kidney rare, and has been estimated to occur once in 2100 to 3000 autopsies. An ectopic pelvic kidney with a huge renal stone in a patient with mullerian agenesis is even rarer. To the best of our knowledge, no such case (ectopic pelvic kidney with stone) has been reported. We report hereby a 40-year-old woman who presented with acute lower abdominal pain and was treated with exploratory laparotomy by a general surgeon under the impression of calcified abdominal tumor. However, the tumor was found to be located in the retroperitoneal space during operation. The urologist took over on the operative table and revealed it to be an ectopic pelvic kidney with urolithiasis associated with the Mayer-Rokitansky syndrome. We describe the clinical course and review the literature of the Mayer-Rokitansky syndrome and associated renal anomalies.