Female form of persistent mullerian duct syndrome: rare entity

ABSTRACT

Persistent Mullerian duct syndrome [PMDS] is a rare form of Disorder of sex development in which Mullerian duct derivatives [fallopian tubes, uterus and the proximal vagina] are present in an otherwise normally differentiated 46 XY male. In the majority of cases, PMDS is a surprise finding either during orchidopexy or during inguinal hernia repair. We report a case of 4 year child with female type [Type III] PMDS. We are discussing the presentation, management and review of the literature