Female form of persistent Müllerian duct syndrome: A rare case report and review of literature

•Persistent Müllerian duct syndrome (PMDS) is a rare form of internal male pseudohermaphroditism.•Diagnosis is often challenging because of lack of familiarity with the condition.•Müllerian duct remnants resection is necessary.•The aims are to preserve fertility and prevent malignant changes. Persistent Müllerian duct syndrome (PMDS) is a rare form of internal male pseudohermaphroditism characterized by the presence of rudimentary Müllerian structures in a virilized male often presenting as undescended testes. Thus, each patient diagnosed with undescended testes should promptly be investigated for PMDS because the early diagnosis has direct effects on outcome and prognosis. A 26-year-old-male complained of long-standing abdominal pain two years ago and was diagnosed having bilateral undescended testes in the pelvic region. He underwent the orchidopexy about one year ago but, after 5 months of orchidopexy, he first complained of discomfort in the left and then right inguinal region due to an incisional hernia that presumed to have the ovotesticular disorder of sexual development. On the pelvic MRI exam, the Müllerian duct structures were observed and he was diagnosed as having PMDS. In this case the patient had bilateral cryptorchidism with testes fixed in the para iliac region with respect to the uterus, indicating the female type of PMDS which is a rare type of PMDS. The case is proven genetically and Müllerian duct remnants have been resected to avoid malignant transformation. Persistent Mullerian duct syndrome (PMDS) is a rare finding and may present as long-standing abdominal pain. Each patient diagnosed with undescended testes should promptly be investigated for PMDS. Diagnosis and management aim to preserve fertility and prevent malignant changes. Therefore, familiarity with this rare condition will lead to adequate management and prevention of complications.