Osteosarcoma is a primary malignant tumor that most commonly affects long bones; however, 10% of cases typically occur in the head and neck. Its histological classification determines the diagnosis of osteosarcoma and the course of treatment. Osteosarcomas of the head and neck primarily affect individuals in their thirties and forties, unlike those of the extremities, which typically occur in adolescents and young adults. Surgical resection is the recommended treatment; it must include negative margins. Chemotherapy and radiation therapy should be used according to the tumor’s histological grade. In summary, treatment should be approached in a multimodal and multidisciplinary manner to improve patient survival and quality of life.