Sacral chordomas are relatively rare, locally invasive, malignant neoplasms. Although metastasis is infrequent at presentation, the prognosis for patients with chordoma of the sacrum is reported to be poor and attributable in most cases to intralesional resection. The value of adjuvant treatment is uncertain, and resection remains the primary mode of treatment. Chordomas are difficult to excise completely, but recent improvements in imaging and surgical techniques have allowed surgeons to perform more frequently en bloc sacral resections with wide surgical margins. The technical challenges of such operations, and the functional costs for the patient (with respect to anorectal and urogenital dysfunction) are significantly increased when the tumor involves high sacral levels. The authors review the clinical presentation and natural history of sacral chordoma and discuss the current treatment techniques and outcomes.