The annual incidence of new cases of osteosarcoma (OS) affects about 0.7 million people, is more frequent in males and in the third and fourth decade of life the average age of highest incidence. Since the involvement of maxillofacial region is rare, around 6.0% of all cases of OS. The aim of this study was to report a case of osteosarcoma in the mandible, describing its clinical, imaging and histopathological. Patient female, 20 years old, leucoderma, searched for referral to a specialized clinic in Surgery and Traumatology Oral and maxillofacial. Increased volume of the anterior mandible region with a history of 3-4 months has been presented in the case below. Surgical excision margin of safety is the main treatment for OS with local recurrence in approximately 60% of patients, usually during the first year after treatment. Survival rates at 5 years are reported with an average of 43% for OS gnathic. It concludes that OS is an aggressive and rare cancer in maxillofacial region, where surgical excision is the best treatment, with high probability of relapse in the first year.