Chordoma is a sporadic type of cancer that affects the spine and is particularly challenging to treat due to the paucity of reported cases and scientific literature. In particular, primary chordomas affecting both the sacral and thoracic vertebrae are extremely rare. We herein report a rare case of chordoma in the sacral and thoracic vertebrae with pulmonary metastasis, along with a literature review. The objective of the present study was to explore treatment options and long-term outcomes in patients with metastatic chordoma. Posterior decompression was performed for the thoracic tumor, followed by extended resection of the sacral tumor. The symptoms of the patient were relieved after surgery, and the postoperative Nurick score decreased from grade 3 to grade 2, while the postoperative McCormick score was I. Therefore, complete chordoma excision and internal spinal fixation may effectively reduce tumor recurrence and metastasis.