Persistent müllerian duct syndrome with transverse testicular ectopia and spermatogenesis

Normal male sexual differentiation is dependent on at least 2 factors: 1) testosterone and 2) müllerian inhibiting factor. The absence of müllerian inhibiting factor is responsible for a rare form of male pseudohermaphroditism, the persistent müllerian duct syndrome or hernia uteri inguinale. Patients with this syndrome present with persistent müllerian structures and the syndrome may be associated with transverse testicular ectopia. Additionally, most patients have azoospermia. We report a case of persistent müllerian duct syndrome with transverse testicular ectopia in which sperm are documented in the ejaculate.