Persistent müllerian duct syndrome: a case report.

The persistent Müllerian duct syndrome is a rare disorder of sexual development. We report on a 4 month-old male who presented with a left-sided inguinal hernia and undescended testes. During the repair of the hernia 2 testes, 1 fallopian tube and an uterus were observed. The clinically suspected diagnosis of hernia uteri syndrome was confirmed by laboratory investigations. At the age of 18 months laparotomy was performed and the 2 fused gonades were descended into the right scrotum.