The literature contains no report on metastatic mixed germ cell tumor at high cervical area. The authors report a patient with metastatic mixed germ cell tumor at craniovertebral junction, who recovered uneventfully after operation. A 31- year-old man was presented with sustained neck pain and slowly progressing quadriparesis. Seventeen years ago, he was operated and received radiation with chemotherapy for the treatment of germinoma on the pineal region, follo- wed by ventriculoperitoneal shunt. Preoperative cervical MRI showed mass lesion at C1-2 area with high signal on T2WI and heterogeneous enhancement on Gd-enhanced T1WI, and syringomyelia at cervicomedullary junction to C7. We performed biopsy based on a patient's past medical history and the laboratory findings: serum AFP and beta- hCG level. On the background of frozen biopsy suggesting mixed germ cell tumor, subtotal resection of the tumor was done with minimal morbidity. Pathologic report confirmed the tumor as mixed germ cell tumor. After operation, the patient recovered uneventfully. We report a rare case of metastatic intramedullary mixed germ cell tumor at high cervical region.