: OBJECTIVES: The authors described a case of rare histological type of primary thyroid tumor-angiosarcoma. Angiosarcoma generally represents a rare connective tissue tumor in the head and neck region. Its biological behavior is locally aggressive and destructive with a high recurrence rate. METHODS: A case of a rare histological type of primary thyroid tumor -angiosarcoma-has been described including fine needle aspiration biopsy, histology, and autopsy. RESULTS: Irrespective of the complex approach the angiosarcoma of the patient referred to was diagnosed in a radically inoperative stage of the disease. Radiation was therefore applied after the tumor mass was diminished with the partial thyroidectomy and a correct histopathological diagnosis was rendered. Nevertheless, the locally destructive course was followed with a rather rapid spread of the tumor (lymph nodes, bones, lungs, and brain). Autopsy proved with certainty that the thyroid was the primary tumor location. A suspected kidney origin due to an uncertain sonography shadow of this region was excluded with a complete absence of any neoplastic spread of the angiosarcoma in the abdominal cavity at all.