Epithelioid hemangioendothelioma (EHE) is an ultrarare vascular sarcoma characterized by epithelioid endothelial cells, whose natural history and treatment are not well defined.[1,2] Histologically, EHE is identified by epithelioid endothelial cells with intracytoplasmic vacuoles and immunoreactivity for vascular markers such as CD31 and FLI-1. This evidence concerns the gene PECAM1 and epithelioid hemangioendothelioma.