Male sexual differentiation in based upon at least two factors; testosterone and the müllerian inhibitor factor. The absence of the latter produces a rare form of male pseudohermaphroditism, the persistence of the Müllerian duct syndrome. The case of a phenotypically male patient in whom surgery to correct bilateral cryptorchidism was performed is presented. The operatory findings included the presence of uterus and fallopian tubes which were removed. The right atrophic tes was removed and the left was fixated. Literature regarding etiology, clinical presentation, diagnosis and treatment is presented.