Persistent Müllerian duct syndrome (PMDS) with seminoma, hernia uterine inguinale, and transverse testicular ectopia is rare. A 41-year-old phenotypic man with 46 XY karyotype was admitted with nonpainful bulging mass in the left inguinoscrotal region. Two distinct masses were identified by sonography and computed tomography. No testis was apparent on the right side. Excisional specimens revealed seminoma and scrotal uterus. Histopathology of seminoma showed enhanced CD117 immunohistochemical staining. The other inguinal mass was consistent with uterine tissue and was associated with the vas deferens in its lateral aspect. Radiation therapy was performed after the orchiectomy and scrotal hysterectomy. The patient remains free of recurrence after 1-year follow-up assessment. The presence of a unilateral inguinal hernia with a large mass associated with a contralateral nonpalpable testis indicates the presence of PMDS with hernia uterine inguinale and transverse testicular ectopia.