Chordoma is rare,locally aggressive malignant neoplasm originated from notochord remnants.Distant extrapulmonary metastases are rare and mostly occur in patient with local recurrence.In this case report,2 years after the radical resection of sacral chordoma,two times successive metastases to the upper limb were diagnosed by immunohistochemistry,without local recurrence.The metastatic tumors were radical excised,and the patient gained radiotherapy postoperatively.More than 2 years after the wide resection of the metastatic focuses,no sign of recurrence and metastasis was detected. Chordoma is rare, locally aggressive malignant neoplasm originated from notochord remnants. Distant extrapulmonary metastases are rare and mostly occur in patient with local recurrence. In this case report, 2 years after the radical resection of sacral chordoma, two times successive metastases to the upper limbs were diagnosed by immunohistochemistry, without local recurrence. metastatic tumors were radical excised, and the patient gained radiotherapy postoperatively. More than 2 years after the wide resection of the metastatic focuses, no sign of recurrence and metastasis was detected.