The clinical features of the giant cell tumor of the sphenoid bone have been discussed and a case report has been added to the fourteen cases reported in the literature. Such cases may first report to an ophthalmologist, an otolaryngologist, a neurologist, or an internist. They should consider this condition in a patient who complains of headache, ocular symptoms such as diplopia, and diminution of vision progressing to complete blindness. The presence of multiple cranial nerve palsies involving II, III, IV, V, and VI nerves in various combinations and the sellar erosion in the lateral x-ray of the skull are quite suggestive of this tumor which should be confirmed by biopsy. The telecobalt therapy appears to give the best results.