Xanthoma is frequently observed in patients with endocrine and metabolic diseases. Intraosseous xanthoma is an extremelyrare lesion. In cases in which associations with systemic metabolic diseases and lipid diseases are ruled out, intraosseousxanthoma is termed primary xanthoma. We herein report an extremely rare case of primary xanthoma of the mandible in a 27-year-old woman. Lower third molar extraction and extirpation of the tumor was performed under general anesthesia following a clinical diagnosis of a benign tumor in the left mandible. Her previous medical history was not contributory, except for ovarian cystoma. A final diagnosis of primary xanthoma was established. Her last follow-up 4 years after surgery showed no sign of recurrence in clinical and radiological examinations
