IntroductionrnEpithelioid hemangioendothelioma (EHE) is an uncommon vascular tumor, which also has been called histiocytoid haemangioma. This low-grade malignant tumor was first described as a distinctive entity in 1982 by Weiss and Enzinger[1]. Histopathologically, EHE is charac-terized by round or spindle-shaped endothelial cells with cytoplasmic vacuolation[2]. The tumor may occur in numerous organs, includingliver, lung, parotid grand, skin, pleura, thyroid, spleen, cardiac valve,radial artery, oral cavity and parapharyngeal space[2-11]. Many cases have been reported, but its etiology and natural history remain un-clear.