A 5-year-old female presented with a history of precocious puberty and increased height. MRI of the brain revealed a nonenhancing hypothalamic mass and associated suprasellar arachnoid cyst. The patient underwent a right frontal craniotomy with orbital osteotomy for wide fenestration of the cyst. Intraoperatively, it was clear the mass represented a hamartoma with a very smooth glistening surface. For this reason, no biopsy of the wall was performed. Postoperatively, the patient remains neurologically intact and without any medication dependence. This case highlights the occurrence of a suprasellar arachnoid cyst in association with a hypothalamic hamartoma.

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