Though osseous abnormalities are associated with tuberous sclerosis, involvement of the skull base and orbit has not been reported. We describe a patient with superior displacement of the orbit and proptosis that were due to sphenoid hyperplasia and sclerosis characteristic of fibrous dysplasia. A pelvic osteosclerotic lesion suggested polyostotic involvement. The bony abnormalities implied neither concurrent neurofibromatosis nor McCune-Albright syndrome in this patient with tuberous sclerosis.