先天性肾上腺皮质增生是女性假两性畸形中较常见的疾病。我们遇到三例。例一,×××,14岁。具男性特征。例二、三是同胞姐妹,8岁及5岁,均有男性体征,阴蒂均增大,阴唇部分融合遮盖阴道口。此三例24小时尿17-酮类固醇含量明显增多,用地塞米松抑制试验后恢复正常,血细胞染色体核型均为46xx。患者均行外阴整形术,切除过长的阴蒂。先天性肾上腺皮质增生属常染色体隐性遗传性疾病,是由于先天性肾上腺皮质内某些酶的缺乏,本文三例属于21-羟化酶不完全缺乏。表现为单纯女性男 Congenital adrenal hyperplasia is a more common disease in female pseudohermaphroditism. We encountered three cases. Example one, × × ×, 14 years old. Male character. Case two, three are siblings, 8 years old and 5 years old, both have male signs, clitoral increases, part of the fusion of the labia majora vaginal opening. The three cases of 24-hour urinary 17-ketosteroid increased significantly, with dexamethasone suppression test returned to normal, the blood cell karyotype were 46xx. Patients underwent genital plastic surgery, removal of the clitoris is too long. Congenital adrenal hyperplasia is an autosomal recessive genetic disease, due to the lack of some enzymes in the adrenal cortex, the three cases belong to the incomplete lack of 21-hydroxylase. Man expressed as a simple male