ABSTRACT
Male pseudohermaphroditism can result from defects in androgen synthesis, androgen action, and mullerian duct regression, and from other uncertain causes. A rare form of male pseudo- hermaphroditism is characterized by the persistence of mullerian derivative in phenotypic male. The retention of mullerian structures can be ascribed to failure of the Sertoli cells to synthesize mullerian duct inhibitory factor, a defect in the response of the duct to that factor, or possibly discordant timing of the release of that factor. We experienced a case of persistent mullerian duct syndrome, which is phenotypically normal male with a right inguinal hernia and a left undescended testis, bilateral fallopian tubes and a uterus. Herein we report a case of early orchiopexy with this syndrome and brief review of the literatures.