Angiosarcoma of the head and neck is a rare and lethal neoplasm. Data from the Connecticut Tumor Registry was analyzed for all head and neck angiosarcomas between 1980 and 2001. The authors also present the first reported case of familial angiosarcoma of the head and neck region. The 1-year mortality was 48% for all patients with angiosarcomas (38% for males, 69% for females). The 5-year survival was 28% for all patients (38% for males, 15% for females). The combination of surgery and radiotherapy resulted in improved survival compared with either treatment alone.