Epithelioid hemangioendothelioma is an uncommon malignant vascular tumor usually involving soft tissue and, in rare cases, the skin. Histologically and biologically it is considered to be a borderline neoplasm between an angiolymphoid hyperplasia with eosinophilia and an epithelioid angiosarcoma. Here we describe an 11-year-old girl with a 1-year history of an isolated, spontaneously appearing, painful ulceration on the instep of the right foot. The histopathologic examination of a wedge biopsy specimen revealed epithelioid eosinophilic cells with intracytoplasmic vacuoles containing erythrocytes. Immunohistochemical staining was positive for the endothelial markers CD31, CD34, and factor VIII. On the basis of these findings the diagnosis of epithelioid hemangioendothelioma was made. Two weeks after complete excision of the tumor, a lymph node metastasis in the right groin was excised. Because of another inoperable lymph node metastasis on the proximal right femur, polychemotherapy was started. As our case report shows, in the event of a nonhealing cutaneous ulceration the possibility of a malignant tumor such as epithelioid hemangioendothelioma should be considered, even in children.