Gender and sexuality in classic congenital adrenal hyperplasia

The current debate about the psychosocial management of intersex patients has significant implications for 46,XX patients with classic congenital adrenal hyperplasia (CAH) in two respects: (1) the question of genital surgery for reasons other than purely medical ones before the patient is old enough to give informed consent, and (2) the question of gender assignment in severely masculinized patients. This article reviews the status of the empirical evidence for the development of gender and sexuality in 46,XX persons with classic CAH and its implications for clinical practice.