We report two cases of 'familial testotoxicosis' in a family of Southern Chinese descent. The proband, an 8-year 4-month-old boy and his 35-year-old father both presented with early sexual development. In both cases the testicular volume was only 6 ml despite fully developed secondary sexual characteristics. Both patients had adult testosterone concentrations but a suppressed gonadotrophin response to gonadotrophin-releasing hormone. The suppressed gonadotrophin response to gonadotrophin-releasing hormone in the father suggests that autonomous gonadal production of sex steroid by the testes can persist well into adult life in some patients with familial testotoxicosis.