Transverse testicular ectopia (TTE) is a rare congenital anomaly in boys, which is characterized by the migrate of both testicles towards the samehemiscrotum or inguinal region. TTE cases are accompanied by the characterized persistent mullerian duct syndrome, which is phenotypically male,with the presence of mullerian duct structures. Persistent Müllerian duct syndrome is caused either by the genetic defect of the anti-mullerianhormone or its receptor. In this case, we reported a TTE associated with persis-tent mullerian duct syndrome in a 12-year-old boy who presentedwith swelling in the right groin and non-palpable left testis. The patient was treated with high ligation of the hernia sac, excision of mullerianstructures and transseptal orchiopexy. In the surgical approach of the cases, we think that removing the mullerian structures as much as possibleand making the testicles palpable without damaging the testicles and other adjacent structures are important.