Giant cell tumors of the skull are very rare and usually occur in the sphenoid bone. The authors report the case of a 10-year-old boy with such a tumor involving exclusively the roof of the left orbit. He presented essentially with edema of the left superior eyelid and diplopia. Computed tomographic examination and magnetic resonance imaging delineated the lesion, which was radically removed via a left fronto-orbital craniotomy. Some aspects of this rare neoplasm are reviewed.