A case of osteogenic sarcoma of the mandible is reported and the literature is reviewed. The patient, a twelve years old boy, complained of pain and swelling of left preauricular region. Blood analysis showed significant leukocytosis. Our tentative diagnosis was inflammation of the parotid gland. Swelling was not decreased in spite of treatment with antibiotics, therefore, tumor extirpation was done under general anesthesia. A massive tumor involved the mandible and centered on the ramus on the left side, extending into soft tissue. Histopathological diagnosis of the frozen section at the time of surgery suggested osteogenic sarcoma. Postoperative irradiation, chemotherapy and immunotherapy were initiated. No evidence of metastasis was found in examination with whole body CT scanning. Local recurrence was found 4 months after surgery, hence, irradiation and multiple drug chemotherapy (VEMP) were again initiated. The patient died of metastasis to the lung. Differential diagnosis in head and neck tumors is important for better prognosis.