a rare case of persistent mullerian duct syndrome and review of literature

Introduction]persistent mullerian duct syndrome (pmds) is a scarce type of male pseudohermaphroditism due to antimullerian hormone dysfunction. so, mullerian duct derivatives such as the uterus, cervix, fallopian tubes, and twothirds of the vagina are seen in a normal phenotypic and genotypic male. considering and assessing the clinical manifestations, associated diseases, histopathology, and imaging findings can be clues in the management of such cases.[case presentation]here, we describe a case of pmds with left inguinal hernia and bilateral cryptorchidism and discuss the management and review of the literature. also, the case has a history of brain mass with a pathologic diagnosis of ganglioneuroma. is there a correlation between pmds and extragenital neoplasm? it needs more investigation in the future.[conclusions]bilateral cryptorchidism could provide us with a hint toward pmds diagnosis. however, genetic counseling will be required, particularly in parental consanguinity.