持續性苗勒氏管症候群乃一罕見之男性偽陰陽症，其特徵為在一外觀正常且體染色體46xy之男性身上存在有子宮及輸卵管等性器官。此症仍源於胚胎其睪丸所分之苗勒氏抑制素（anti-Müllerian hormone）製造或作用失常所致。本文報告一遺傳表型正常之年輕男性，于接受左側腹股溝疝氣併右側隱睪症手術時，意外發現其體內存在有一子宮與兩條查輸卵管等苗勒氏構造。因此惡性轉化之可能，故所有與男性不符之器官與腹腔內睪丸均予以切除。除了因長期隱睪造成的病理變化外，並無其它異常之組織學發現。染色體檢查顯示正常的46xy核型，而確立此持續性苗勒氏管症候群之診斷。提出此病例並回顧相關文獻報告。

Persistent Müllerian duct syndrome (PMDS), a rare form of male pseudohermaphroditism, is characterized by presence of a uterus, cervix, and Fallopian tubes in an otherwise normally differentiated 46 XY male, caused by a defect in the synthesis or action of the inhibitory hormone of the Müllerian ducts secreted by the fetal testis. A case is reported of a phenotypically normal male with persistent Müllerian structures consisting of a uterus and two Fallopian tubes. The unusual structures were found accidentally during surgery for left inguinal hernia and right cryptorchidism. All the structures contrary to the assigned male gender and the intraabdominal testis were removed for possible malignant potential. No pathological abnormalities apart from those associated with prolonged cryptorchidism were found in the Müllerian structures and the testicle. The chromosome study revealed normal 46 XY karyotype, which confirmed the diagnosis of RMDS. The pathogenesis, diagnosis and management of this syndrome are reviewed briefly.