Persistent Mullerian duct syndrome in adult: a case report

A twenty-eight year old phenotypically and genotypically normal male adult was admitted with a right inguinal hernia, a right retractile testis and left cryptorchidism. During surgery, he was found to have a uterus in the right spermatic cord and a left undescended testis (intra abdominal type). The uterus was excised, left orchidectomy, right orchidoplexy and right herniorrhaphy were performed. This rare case is reported as persistent Mullerian Duct Syndrome (PMDS) which is caused by impaired action of Mullerian Inhibiting Substance (MIS) in regressing the Mullerian duct.