Aspects of 5-alpha reductase deficiency, a review

Two forms of 5 alpha-reductase deficiency have been described and two genes have been cloned. In view of the psychoendocrinological complexity of the primary form, the early diagnosis preferably in infancy, is crucial. Rearing up those who are assigned as females to the male gender identity could minimize the risk of gender identity and role disorders when puberty is reached.