The authors describe a case of a 35-year-old man with 5-year duration infertility. History, clinical examination, and laboratory tests have established the diagnosis of late-onset congenital adrenal hyperplasia, due to 21-hydroxylase deficiency. Treatment with dexamethasone resulted in improvement of sperm quality, and 4 months later a pregnancy was achieved. Two years after the patient fathered his first child, and while he had discontinued dexamethasone treatment, he succeeded at a second pregnancy. The authors conclude that (1) late-onset congenital adrenal hyperplasia presents with significant variation during the patient's lifetime; (2) glucocorticoid administration is necessary in infertile men with nonclassic form of 21-hydroxylase deficiency; and (3) in cases of male infertility of unknown origin, the patient must be scrutinized for congenital adrenal hyperplasia, especially the nonclassic form.

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