Epithelioid hemangioendothelioma is a low-grade malignant tumor with a histologic appearance and clinical course between that of a hemangioma and angiosarcoma. It is rarely encountered in the bone. A 48-year-old woman was examined following trauma. A cystic lesion was noted on a plain radiograph of the left foot, destructing the diaphysis of the first metatarsal bone. Magnetic resonance imaging showed a solid intramedullary lesion involving a large part of the bone. Scintigraphic examination showed uptake in the diaphysis of the left tibia and the first metatarsal bone of the left foot. Histopathologic examination showed a neoplastic lesion consisting of atypical endothelial cells lining vascular structures or forming solid nests in a myxoid stroma. The tumor was immunoreactive for factor VIII, CD31, CD34, and vimentin. A diagnosis of epithelioid hemangioendothelioma was made and the patient underwent subtotal resection of the metatarsal bone with reconstruction of the fibula, and a wide resection of the tibial lesion. No recurrences or metastasis were observed during a four-year follow-up.