Precocious puberty in a male with Prader-Labhart-Willi syndrome

True precocious puberty is reported in a male child with Prader-Labhart-Willi syndrome. The diagnosis of precocious puberty was made at 8 6/12 years of age when a spontaneous migration of previously unpalpable testes (mean volume = 5 ml), an increase in penis length (from 2 to 6 cm) and a growth spurt (8.4 cm/year) occurred. The follow-up until 13 years of age (bone age 15 years) showed a progressive pubertal development, facial acne (10 years), frequent spontaneous erections and ejaculations (11 years). Repeated endocrine and neuroradiological investigations were consistent with an idiopathic form of precocious puberty.