Ambiguous genitalia require prompt decision in the neonatal period to give the child the best opportunity to avoid later major problems of sexual identification. A team approach including the neonatologist, the pediatric endocrinologist, the geneticist and the pediatric urologist seems to be the best way to make the right decision. This paper is a review of 48 cases of ambiguous genitalia referred to our center for management. The diagnosis of female pseudohermaphroditism was established in 30 cases with 21 presented with adrenogenital syndrome. Ten patients were considered as having a diagnosis of male pseudohermaphroditism, six presented with gonadal dysgenesis and two were true hermaphrodites. Principles of surgical management for each category of patients is exposed, discussed and will be the way to treat subsequent cases.
