Objective: To review the presenting signs and symptoms of patients with clival chordomas. Methods: A retrospective study of 18 patients with pathologically proven clival chordomas evaluated and treated at University of Arkansas for Medical Sciences from 1991 to 1998. Two of the 18 patients had careful neuro-ophthalmologic examination. Results: The most common symptoms at presentation included diplopia (66%) and headache (50%), while the most common neuro-ophthalmic sign was a sixth cranial nerve palsy (44%). At least one patient had resolution of his symptoms after surgery. Conclusions: Clival chordomas most commonly present with diplopia, usually from a sixth cranial nerve palsy.