Epithelioid hemangioendothelioma (EH) is a unique vascular tumor characterized histologically by epithelioid endothelial cells. A case of EH arising from the dermis and presenting as a nodule of the palm is described. To our knowledge, this is the third report of a cutaneous presentation of EH and the first report of a patient with this rare neoplasm with solitary cutaneous involvement. A review of the literature is also presented.