Persistent Mullerian Duct Syndrome with Malignant Conversion of Mullerian Remnant

 A case report of a very rare entity of persistent Müllerian duct syndrome with malignant transformation of Mullerian remnant is presented. A twenty-two-year-old phenotypically normal male presented with complain of hematuria and lower abdominal pain. On examination patient had bilateral cryptorchidism and on imaging bilateral duplication of pelvicalyceal system and ureters and left sided ureterocoele. There was a malignant mass lesion with metastatic lymphadenopathy in pelvis. The diagnosis of persistent Mullerian duct syndrome was suspected on MRI pelvis and confirmed by genetic and pathological testings. Risk of malignant transformation of undescended testis and Mullerian remnants increases with age and have impact on psychological health of patient, so early diagnosis becomes crucial.