A giant cell tumor involving the vertex of the skull is described in a 3-year-old child with no history of head trauma. The mass was present approximately 4 months prior to resection. Microscopically, the lesion consisted of highly cellular tissue composed of oval to spindle-shaped stromal cells admixed with numerous multinucleated giant cells. Giant cell tumor of the skull is a rare lesion, usually involving the sphenoid or temporal bone in adults. The differential diagnosis is discussed with reference to the literature regarding giant cell lesions, especially of the cranium. The authors are unaware of previous reports of a similar lesion in this location in such a young child.