PERSISTENT MULLERIAN DUCT SYNDROME

We report an 18-month old male presenting with a right-sided inguinal hernia and undescended testes. At herniotomy, a uterus and two fallopi an tubes were found in the pelvic peritoneum adjacent to the two gonad s which received their blood supply partly along the Mullerian duct re mnants. The gonads were testes by histological examination. Bilateral orchiopexy was performed without removal of the Mullerian structures f or fear of jeopardizing the testicular blood supply. The diagnosis of persistent Mullerian duct syndrome was confirmed postoperatively by ge netic and hormonal investigations.