Chordomas are uncommon tumors arising from embryonic remnats of the fetal notochord. The spheno-occipital and sacrococcygeal regions account for over 90 per cent of cases, the remaining 10 per cent occurring anywhere along the vertebral axis. A case of chordoma arising from the clivus is presented with a view to discussing management. The ability to obtain reasonable survival times associated with a useful and meaningful quality of life is emphasized. This may be accomplished by repeated, judicious surgical intervention with surprising ease and little morbidity to the patient in spite of its intracranial location.
