Reports regarding osteosarcoma of the maxilla are rare. Craniofacial osteosarcoma accounts for approximately 1% of all head and neck neoplasms and occurs predominantly during the 3rd and 4th decade of life. Histologically, osteosarcoma can be divided into four major types, each presenting with its own particular features. The development of osteosarcoma has been reported to be associated with risk factors, such as exposure to radiation, Paget's disease, fibrous dysplasia, and retinoblastoma. Surgical resection is the treatment of choice. Osteosarcoma with intracranial invasion has a high recurrence rate (80%) and is also associated with a high mortality rate. However, considerable improvement in survival can be achieved by chemotherapy. Known prognostic factors include tumor sites, metastasis and Padget disease. In this report, we presented an 18-year-old male patient with maxillary osteosarcoma. After treatment by us, he has been disease-free for 6 year and is still receiving regular follow-up. In this case report we want to share our positive experiences with this patient and the treatment of osteosarcoma as well as reviewing previous studies.