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.