We report a case of chordoma with minimal metastasis to the lung in a 56-year-old female suffering from disfunction of borh upper and lower extremities and from the pain in the nuchal area and upper extremities. The tumor was found on the right posterior root segment of the eigth cervical vertebra at the first operation, and to be located anteriorily between the fifth cervical and first thoracic vertebrae at the second opration. The autopsy disclosed tumor masses (10.0×3.5×5.0cm) located anteriorily between the fourth cervical and second thoracic vertebrae separated into three lobules by thin fibrous tissue. In addition, the tumor extended to both yellow and posterior longitudinal ligaments, invading the seventh cervical vertevra. The tumor showed typical characteristics of chordoma microscopically as well as histochemically. Although the possibility that the tumor originated from the intervertebral disc could not be completely excluded the tumor was considered to originate from the notchordal remnant of the seventh cervical vertebra.