苗勒管永存综合征(persistent Müllerian ductsyndrome,PMDS)是一种少见的男性假两性畸形,其特点是存在苗勒管结构或苗勒管退化不全产物,而且基因型都是正常男性。睾丸横过异位(trans-verse testicular ectopia,TTE)是先天性双侧睾丸通过一侧腹股沟管下降,降于腹股沟或位于阴囊的一种先天畸形。PMDS通过门诊问诊及查体很难诊断,往往通过手术探查才能发现。本文通过2例PMDS病例来探讨PMDS及TTE的发病机制、临床 Persistent Müllerian ducts syndrome (PMDS) is a rare male pseudohermaphroditism characterized by the presence of Müllerian ductal structures or Müllerian ductal degenerative products, and the genotypes are all normal males. Trans-verse testicular ectopia (TTE) is a congenital malformation of congenital bilateral testis descending through the groin on the side of the groin, down to the groin, or in the scrotum. PMDS through outpatient clinics and physical examination is difficult to diagnose, often through surgical exploration can be found. In this paper, two cases of PMDS to explore the pathogenesis of PMDS and TTE, clinical