Persistent Müllerian duct syndrome is a rare form of internal male pseudohermaphroditism. The condition is characterized by cryptorchidism and retention of Müllerian derivatives (uterus, fallopian tubes, and upper vagina). We report the case of a 29-year-old man with this syndrome, who presented for workup of infertility. The condition is associated with a high progesterone level. We provide the clinical details of this case as well as the pathogenesis of persistent Müllerian duct syndrome, along with a review of the literature.