This case illustrates a rare instance of persistent Müllerian duct syndrome (PMDS) in males, also known as anti-Müllerian hormone or Müllerian inhibiting substance deficit, which results in male pseudohermaphroditism (MIS)

This syndrome is characterized by the persistence of Mullerian duct derivatives (i.e. uterus, cervix, fallopian tubes and upper two thirds of vagina) in a phenotypically and karyotypically male patient.

PMDS has been described to have two anatomic variants:

• male form (80-90% of cases) characterized by the presence of unilateral cryptorchidism and contralateral inguinal hernia. It's subdivided into 2 types according to the contents of the hernia sac

  • Hernia uteri inguinali, containing undescended testes, uterus and unilateral fallopian tube

  • crossed testicular ectopia containing both testes, uterus and both fallopian tubes

• female form (10-20% of cases)

DDx includes mixed gonadal dysgenesis, which is a disorder of sex disease occurs secondary to the dysfunction of both Leydig and Sertoli cells. It manifests with external genital ambiguity, which is not seen in patients with PMDS