Treatment of upper cervical chordoma is generally considered to be difficult one, because of serious troubles in supporting cranio-spinal junction after the surgery and at the recurrence. Clinical course of a 22-year-old man with chordoma originated from C2 vertebral body was reported, and the operation for such a condition were discussed with emphasis on the mode of craniovertebral fixation. Simple subtotal removal of the vertebral chordoma was carried out by posterior approach at the first operation. The second one was necessary fourteen months later for recurrence of the tumor. At the second surgery, in addition to the tumor removal, posterior fusion between the occipital bone and the C4 lamina was performed with homologous iliac bone graft, the center of which was kept slightly lateral to the lesion. This procedure of fixation not only enabled a sufficient removal of the residual tumor without loosening the bone graft, but also shortened the bedridden time following the subsequent operations for the recurrence, and finally resulted in an excellent recovery in his clinical symptoms. It is considered to be important to select the most adequate approach of surgery, including fixation technique, according to the extension of the tumor. We would emphasize the usefulness of paramedian posterior fusion of the cranio-spinal junction for such a condition of wide tumor invasion in the high cervical region.