Chordomas are the most common primary malignant tumor of the mobile spine and of the sacrum. Although considered not to possess significant metastatic potential, such lesions are locally aggressive, leading to neurologic compromise and lytic destruction of bone. En bloc resection has afforded patients the greatest chance of local control and disease-free survival. Such radical resections may be associated with significant surgical morbidity, however. Although considered generally resistant to radiation therapy and chemotherapy, recent advances in photon and proton radiation therapy and use of monoclonal antibodies may provide improved outcomes for poor surgical candidates and for tumors that recur after surgery.