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Chordomas are midline tumors of notochordal origin, occurring anywhere from the skull base to the coccyx. Although one-third of chordomas occur in the sphenooccipital region, to our knowledge only 1 case of jugular foramen chordoma with unusual extension into the neck has been reported in the literature to date. A 21-yearold woman presented with a 3-year history of a large neck mass and partly compensated lower cranial nerve symptoms of insidious onset. Imaging revealed a tumor involving the posterior cranial fossa and carotid space, with widening and erosion of the jugular foramen. Characteristic histopathologic findings and immunohistochemical staining confirmed the diagnosis. The tumor was removed by a combined retrosigmoid and lateral cervical approach. The patient was disease free 18 months after treatment. [ABSTRACT FROM AUTHOR]