A 15-year-old boy presented with a history of medically refractory gelastic seizures and cognitive impairments. Neuroimaging demonstrated a sessile type hypothalamic hamartoma, which was treated by gamma knife surgery. However, the gelastic seizures only partially decreased and the frequency of seizures remained unchanged. One year later, angiography before surgery detected anterior paraclinoid aneurysm of the left internal carotid artery. Blood pressure and endocrinological examinations showed no abnormality. Direct surgery was performed to treat the aneurysm and hamartoma. No sclerotic changes were noted in the arterial wall. The aneurysm was treated with clipping, and the hamartoma was partially removed. Postoperative course was uneventful and the gelastic seizures disappeared. No evidence for a causal relationship between the hamartoma and aneurysm was found.