Introduction: Hypothalamic hamartomas are associated with precocious puberty, gelastic seizures and severe refractory epilepsy. Treatment options include surgical resection, radiofrequency and radiosurgery. Case Report: A 7-month-old girl presented with gelastic seizures and developmental delay related to a giant hypothalamic hamartoma. The patient was operated through a subfrontal approach. Intraoperatively the lesion appeared intimately adherent to the right internal carotid artery. Seizure control was improved after tumoral decompression. Conclusions: Treatment of giant hypothalamic hamartomas should always include surgical resection, given the mass effect over surrounding vital structures. Subfrontal approach with orbitary rim osteotomy provides a wide exposure with minimal frontal lobe retraction. Close adherence of hypothalamic hamartoma to vascular structures may be present, requiring careful surgical manipulation.

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