Persistent Müllerian Duct Syndrome: a Case Report

Abstract

Persistent Müllerian duct syndrome is a rare form of internal male pseudohermaphroditism caused by defects in synthesis or action of Müllerian-inhibiting factor, due to which Müllerian duct derivatives, such as uterus, fallopian tube, and upper vagina, are normally present in 46XY males. Here, we report a 26-year-old male with right-sided obstructed inguinal hernia with left undescended testis. On exploration, hernial sac containing bowel loops, uterus with fallopian tubes, upper vagina, and testes were present.