Chordomas arising from the low cervical spine have been reported rarely. This case report expands the knowledge of chordomas of the low cervical spine by reporting the clinical, radiological, and pathological findings of a case of chordoma arising from C6 vertebra in a 61-year-old man presenting with left Horner's syndrome. This clinical presentation is uncommon for cervical chordomas and resulted from the tumor growth in the left carotid space resulting in the total encasement of common carotid artery and involvement of sympathetic fibers ipsilaterally.