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Hypothalamic hamartoma (HH) is a rare developmental malformation often characterized by gelastic seizures.Recent advances in treating HH have led to dramatic improvements.However,clinical protocol of HH is poorly understood.Since 2002,department Pediatric Neurosurgery of Xinhua Hospital has maintained a multidisciplinary clinical program to evaluate and treat children with HH.This program has provided the opportunity to investigate the management of HH.In this review,we summarize the clinical progress and propose a clear management principle for different HH patients.