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.