Persistent müllerian 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 fallopian tubes were found in the pelvic peritoneum adjacent to the two gonads which received their blood supply partly along the müllerian duct remnants. The gonads were testes by histological examination. Bilateral orchiopexy was performed without removal of the müllerian structures for fear of jeopardizing the testicular blood supply. The diagnosis of persistent müllerian duct syndrome was confirmed postoperatively by genetic and hormonal investigations.