RESUMEN
Hypothalamic hamartomas are benign turnouts that are often associated with central precocious puberty. Resection of hypothalamic hamartoma has been recommended as a treatment option for selected cases of pedenculated lesions, especially in young children. We report a case of a four-year-old boy whose onset of puberty began at approximately 10-months of age when his parents noticed incipient pubic hair and a husky voice. Magnetic resonance imaging demonstrated a pedenculated isodense mass below the tuber cinereum associated with a pituitary microadenoma. The hamartoma was totally removed microsurgically. Postoperatively, the signs and symptoms of precocious puberty began to disappear. Clinical presentation, mechanism and treatment of precocious puberty caused by hypothalamic hamartoma are fully discussed