Epithelioid hemangioendotheliomas are unique vascular tumors characterized by epithelioid or histiocytoid endothelial cells that mainly affect adults. This low-grade malignant vascular tumor was described as a distinctive condition in 1982 by Weiss and Enzinger. Although the tumor is classified in between an angiolymphoid hyperplasia with eosinophilia and an epithelioid angiosarcoma, it sometimes takes a clinical course resembling that of angiosarcoma. We describe that case of a 12-year-old boy who presented with an approximately 6-month history of a spontaneous chronic lesion in his right armpit and became a painful ulceration in the prior 2-month period. The histopathologic examination revealed small nests and cords of spindling epithelioid endothelial cells, intracytoplasmic lumina containing erythrocytes, pinocytotic vesicles and a necrotic area. Immunohistochemical staining was positive for the endothelial markers CD31, CD34, CK(+), and vim(+). On the basis of these findings the diagnosis of EHE was made. After surgery, pathologic examination revealed metastasis in the lymph nodes. So polychemotherapy was started. As our case report shows, it is possibility that cutaneous ulceration of a malignant tumor such as EHE should be considered, even in children.