Epithelioid angiosarcoma of the thyroid is a rare mesenchymal neoplasm, mainly described in the Alpine area. This lesion was initially considered an anaplastic carcinoma, but immunohistochemical and ultrastructural features have proven the endothelial differentiation of tumor cells. In fact, the peculiarity of this tumor concerns the expression of low-molecular weight cytokeratins by neoplastic cells of endothelial origin. We describe a case of epithelioid angiosarcoma of the thyroid occurred in a 73-year-old woman from an Italian non-Alpine area, and a review of non-Alpine primary thyroid epitheliod angiosarcoma is presented.