Extraosseous osteosarcoma arising in the head and neck region is an exceptional finding, with a few isolated cases informed in the literature. We report a new case of a large high-grade extraosseous osteosarcoma located in the neck.: A 74 year-old woman with a tumor on the right side of the neck was treated by complete surgical resection. The histopathological diagnosis was osteoblastic extraosseous osteosarcoma. PET-CT scan disclosed no evidence of disease in other areas. Patient received adjuvant radiotherapy and after 3 months of follow-up tumor activity was detected in the cavernous sinus and the patient died of disease one month later.Extraosseous osteosarcoma in head and neck region is exceptional, and incisional biopsy is the only method to establish a diagnosis and the one to differentiate it from other bone-producing soft tissue tumors. Surgery is the gold standard for treatment of extraosseous osteosarcoma.