Persistent Mullerian duct syndrome is a rare form disorder of sexual differentiation characterised by the persistence of Mullerian derivatives (fallopian tubes, uterus and the proximal vagina) in males with an XY karyotype and normal virilisation. We report a case of a 29-year-old man with right transverse testicular ectopia, mix germ cell cancer at ectopic right testis and left-sided obstructed inguinal hernia containing a uterus and fallopian tube. We performed orchiectomy and hysterectomy on the patient.