Giant cell tumor of the skull is a rare clinical entity. This report presents the case of a 32-year-old female with Turner's syndrome and a giant cell tumor of the left middle cranial fossa floor. Although there have been sporadic reports of patients with extragonadal tumors, to our knowledge this is the first reported case of giant cell tumor in a patient with Turner's syndrome. Definitive treatment is surgical extirpation; however, recommendations for cases of subtotal excision remain controversial. Management of this patient with giant cell tumor of the skull is described, and current treatment recommendations are discussed.