Epithelioid hemangioendothelioma (EGE) is a rare locally invasive neoplasm with metastatic potential. It most commonly occurs in the liver, lungs, and bones, but it can also be present in many other organs, including the nasal cavity. Depending on the volume and location in the nasal cavity, EGE can be manifested by difficulty in nasal breathing of varying severity, epistaxis, and mucous discharge from the nose. Computed tomography of the paranasal sinuses, endoscopic examination of the nasal cavity do not allow differentiating this type of tumor due to the lack of specific signs. The decisive role in the diagnosis of EGE belongs to the immunohistochemical study of the tumor. The main treatment is surgical endonasal removal of the tumor followed by chemoradiotherapy if there are signs of malignancy according to the results of immunohistochemical studies.