DOI: 10.36347/sjams.2018.v06i07.007

Pages: 2647-2650

Congenital adrenal hyperplasia (CAH) by 11-Beta-hydroxylase deficiency is a rare autosomal recessive disorder that accounts for 5-8% of CAH. We report the observation of one CAH discovered during a sexual development disorder with severe hypertension resistant to treatment in a girl aged 15 years, raised as a girl. A feminization surgery was proposed, a feminizing genitoplasty was performed without adrenalectomy because of the correction of blood pressure figures under medical treatment. Late diagnosis of CAH poses a problem of sexual orientation, so antenatal diagnosis and treatment are essential to avoid virilization of the female fetus.