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Persistent Müllerian duct syndrome (PMDS) is an unusual form of internal male pseudohermaphroditism in which Müllerian duct derivatives are seen in phenotypically normal males, with 46, XY karyotype. PMDS is an outcome of defective functioning of the Müllerian inhibiting factor (MIF) or its receptor. MIF causes degeneration of the Müllerian duct in the fetus. PMDS clinically manifests as cryptorchidism or inguinal hernia, although it is mostly undetected until puberty. We report a rare case of PMDS in a 45-year-old male patient with right-sided cryptorchidism and contralateral inguinal hernia. Upon exploration of the hernial sac, it contained a uterus-like mass with attached left testis. Biopsy and subsequent histopathological examination of the mass confirmed the presence of endometrial glands.