Abstract: Congenital adrenal hyperplasia (CAH), an autosomal recessive disease, is characterized by the adrenocortical steroid hormone synthesis disorder. The most common type is 21-hydroxylase deficiency. This disease is generally accompanied with male infertility, which is mainly due to adrenal residual tumors and the change of gonadotropins. The diagnosis of CAH is often missed due to its rarity and atypical symptoms. The early diagnosis and treatment in childhood can improve the reproductive function of male patients in adults. CAH is mainly treated with glucocorticoid, and some of adult male patients can restore their fertility. For those male patients who can not restore their natural fertility, the assisted reproductive technology can be recommended. Meanwhile, if the diagnosis of CAH in adults was definite, the sperm cryopreservation should be done as soon as possible. In this article, we report a case of CAH with male infertility and provide a short review.

Key words: Adrenal cortex diseases, Adrenal hyperplasia, congenital, Infertility, male, Therapy, Fertilization in vitro