Abstract: Objective: Osteosarcoma of jaw (OSJ) is a rarely seen tumor with a very high malignancy rate. The aim of our article is to investigate the treatment and prognosis of OSJ and to assess the prognostic factors of OSJ patients. Methods: The clinical data of 26patients with OSJ, who were admitted to our hospital during a period from 1999 to 2009, were retrospectively analyzed. There were 15male and11female patients, with a male to female ratio of 1.36:1. The age of on -set ranged from11to 66years (mean age 36.2). Of the 26patients, 14patients (53.8%) suffered OSJ with the primary fo-cus in the maxilla and the other12patients (46.2%) in the mandible. Of all the cases,8 cases received radical simple sur -gery only (SS) and 18cases received combined therapy (CT) of surgery plus postoperative radiotherapy and / or chemo-therapy. Kaplan-Meier survival curve and COX regression analysis were used to analyze the clinical data and the Enter method was used for verification. Results: The overall 5-year survival rate was 41.0% for all 26patients, with a relapse rate of 76.9% (20/26). In 18of the 20relapse cases ( 90%), the relapse occurred within two years after surgery. The 5-year sur -vival rates were37.5% and 43.1% in the SS and CT groups, respectively, without significant differences between the two groups (P> 0.05). The relapse rate was 87.5% (7/8) in the SS group and 72.2% (13/18) in the CT group, without significant differences between the two (P>0.05). Multivariate COX regression analysis indicated that the pathological grading is the in-dependent influencing factor for mortality of the patients. Based on this, the death risk is obviously higher in the patients with poor and moderate differentiation compared to those with well differentiation. Sex, diseased region, pathologic types and mode of treatment are not independent factors affecting the prognosis. Conclusion:Radical surgery is the main treat -ment of OSJ. Adjuvant radio-chemotherapy can be considered as a treatment choice in OSJ patients who fail the thorough-going surgery. However, further studies are still needed to verify the therapeutic efficacy. Satisfactory prognosis can be achieved in the cases with well differentiated OSJ.