[Hernia inguinalis uteri in the male]

Persistent Müllerian duct syndrome (also known as uterine inguinal hernia) is described and two cases are reported. In this rare type of male pseudohermaphroditism there is no disturbance of either primary or secondary external masculinization. Consequently, this anomaly is always diagnosed incidentally, generally at operation for cryptorchidism or inguinal hernia. In addition to bilateral normal testes, these patients possess a uterus with two uterine tubes. The persistence of the internal female genitalia is caused by the insufficiency of the MIF hormone (Müllerian Inhibiting Factor). Early orchiopexy is recommended. Removal of the female internal genitalia is seldom required.