Pregnancy terminated for a severe oligoamnios and renal dysplasia. Chromosomal, gonadal, internal genitalia sexes are female. There is a cloacal dysgenesis, with caudal appendice and hypoplastic external genitalia of male type. Single umbilical artery and congenital cardiac malformation (complete atrio ventricular communication) are associated. Embryopathologic explanation for this female pseudo-hermaphroditism is proposed.