Hypothalamic hamartoma is a rare congenital nonneoplastic heterotopia consisting of neurons, glial cells and fiber bundles. Clinically, most patients with hypothalamic hamartomas present with precocious puberty and/or gelastic epilepsy. We report an interesting case of hypothalamic hamartoma causing precocious puberty in a young male. The lesion was excised totally through frontotemporal craniotomy and transSylvian approach without any added morbidity.

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