Abstract
A 16-month-old boy who presented with gelastic seizures and central precocious puberty due to hypothalamic hamartoma is presented. Focal seizure activity with secondary generalisation was documented by the electroencephalogram. The condition was partially controlled by carbamazepine. The central precocious puberty, which was confirmed biochemically by a brisk pubertal rise of gonadotropin in response to leutenising hormone-releasing hormone, was also controlled by a LHRH long-acting analogue. The literature and the current controversy in the management of this condition are reviewed.