Chordoma is a very rare, poorly known malignancy, with slow progression, mainly located in the sacrum and spine. All age groups may be affected, with a diagnostic peak in the 5th decade of life. Clinical diagnosis is often late. Histologic diagnosis is necessary, based on percutaneous biopsy. Specific markers enable diagnosis and prediction of response to novel treatments. New radiation therapy techniques can stabilize the tumor for 5 years in inoperable patients, but en-bloc resection is the most effective treatment, and should be decided on after a multidisciplinary oncology team meeting in an expert reference center. The type of resection is determined by fine analysis of invasion. According to the level of resection, the patients should be informed and prepared for the expected vesico-genito-sphincteral neurologic sequelae. In tumors not extending above S3, isolated posterior resection is possible. Above S3, a double approach is needed. Anterior release of the sacrum is performed laparoscopically or by robot; resection uses a posterior approach. Posterior wall reconstruction is performed, with an associated flap. Spinopelvic stabilization is necessary in trans-S1 resection. Total or partial sacrectomy shows high rates of complications: intraoperative blood loss, infection or mechanical issues. Neurologic sequelae depend on the level of root sacrifice. No genital-sphincteral function survives S3 root sacrifice. Patient survival depends on initial resection quality and the center's experience. Immunotherapy is an ongoing line of research.