Cranial base chordoma, a rare, usually slow-growing tumour of bone thought: to arise in notochord remnants, presents itself when its growth in the spheno-occiput results in cranial nerve compression or an obvious nasopharyngeal mass. The present review of 11 cases of cranial base chordoma encountered at three McGill University teaching hospitals from 1979 until 1992 examines presentation, radiologic findings, pathology, treatment, and outcome. A literature review surveys chordoma with regard to epidemiology, diagnosis, efficacy of medical and surgical modalities on tumour control, and survival. Discussion focuses on current therapeutic options relative to histopathologic characteristics of chordoma, and whether or not these may be used to predict course of disease and outcome.