Epithelioid hemangioendothelioma described first by Weiss and Enzinger in 1
982 is an uncommon vascular tumor usually involving soft tissue, less frequ
ently the lung and the liver and exceptionally the skin. We herein report a
52-year-old woman who presented an isolated moderately painful persistant
ulceration of the concha of her left ear, Histopathological findings showed
strands and nests of epithelioid endothelial cells typical of cutaneous ep
ithelioid hemangioendothelioma. Immunohistochemical stainings confirmed the
vascular nature of the tumor. Surgical excision by ear amputation was perf
ormed. In a review of the literature, to our knowledge, this clinical prese
ntation as ulceration has never previously been reported.