Persistent Müllerian duct syndrome is associated with cryptorchidism and transverse testicular ectopia. Such gonads are at an increased risk of malignant transformation. Furthermore, most patients have azoospermia. Here in we report about two brothers with persistent Müllerian duct syndrome. The diagnosis was made during surgical operation for testicular cancer in younger brother. In the other one, persistent Müllerian duct syndrome was diagnosed during examination for infertility.