An epithelioid hemangioendothelioma (EHE) is a rare vascular tumor of the head and neck region, which carries a risk of recurrence and metastasis. Its etiology is still unclear. It frequently involves the lungs, liver, and bones. Rarely, it may manifest in other parts of the body. Pathologic immunohistochemical investigations are essential to make the definite diagnosis. As it is uncommon, there is no standardized protocol for the treatment of EHE. Herein, the authors report a rare patient with of an EHE localized in the head and neck region.