Abstract

The persistent mullerian duct syndrome (PMDS) is a relatively rare form of internal male pseudohermaphroditism. In this type of male pseudohermaphroditism, there is no disturbance of either primary or secondary external masculinization. Consequently, this anomaly is mostly diagnosed incidentally, generally at operation for cryptorchidism or inguinal hernia. Here, we report a case of 30-year-old patient, an otherwise normal male, presenting with mullerian duct derivatives such as uterus, ovary, fallopian tube, and proximal vagina in the sac of left-sided irreducible inguinal hernia with right-sided undescended testes.

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