The typical, well recognized childhood epilepsy syndrome caused by hypothalamic hamartoma is characterized by earls-onset, stereotyped attacks of uncontrollable laughter, frequent refractors seizures with progressive cognitive deterioration and severe behavioral problems. Here, we report a 17-year-old patient with.,elastic phenomenon started in the neonatal period, later on associated with drug resistant polymorphic seizures, intellectual deficit and behavioral disorders, who improved by partial resection of an expected hypothalamic hamartoma that, in turn, resulted to be a hypothalamic low-grade astrocytoma. (C) 2002 Elsevier Science B.V. All rights reserved.