Introduction: Persistent Mullerian duct syndrome is a rare form of male pseudo-hermaphroditism characterized by the presence of Mullerian duct structures in an otherwise phenotypically, as well asgenotypically, normal man; only a few cases have been reported in the worldwide literature. A greatvariety of organs have been found in indirect inguinal hernial sacs.

Case presentation: We report a case of 28 year old man, father of 2 children with unilateral cryptorchidism on the left side and testis and painful left groin mass. we found uterine tissue extending through the inguinal canal.

Conclusions : PMDS is a rare form of male pseudo-hermaphroditism characterized by the presence of Mullerian duct structures in an otherwise phenotypically, as well as genotypically, normal man. Hernia uteri inguinalis is type I of the male form of PMDS, characterized by one descended testis and the herniation of the ipsilateral corner of the uterus and fallopian tube into the inguinal canal.

Keywords: mullerian duct syndrome, cryptorchidism