Persistent mullerian duct syndrome is a relatively rare inherited defect of sexual differentiation characterised by failure of regression of the mullerian ducts in males. In affected individuals, the uterus and tubes are present due to a defect of synthesis or action of antimullerian hormone normally produced by testicular Sertoli cells. The authors report four cases with a mean age of 20 years. All patients were phenotypically normal males, with bilateral cryptorchidism in two cases and unilateral irreducible inguinal mass in the other two cases. The mullerian ducts were removed in two cases and left in place in two cases as they were intimately adherent to the vas deferens. Long-term follow-up was decided due to the risk of malignant transformation of these remnants recently described in literature. According to the authors, the best management of these structures is excision and orchidopexy. In the case of adult infertility with testicular atrophy, gonadectomy and androgen replacement therapy are recommended.
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