Epithelioid hemangioendothelioma is a rare tumor, originating from the endothelium of blood vessels, that occupies an intermediate place between hemangioma and angiosarcoma. Epithelioid hemangioendothelioma refers to malignant tumors along with angiosarcoma. Epithelioid hemangioendothelioma is localized in the skin, in soft tissues, liver, lungs, heart, less often in bones. The bone form accounts for about 6% of cases. The article presents a case from practice with a description of the X-ray picture of epithelioid hemangioendothelioma of the femur. The difficulties of differential diagnosis are presented. The presented observation will improve the diagnosis of this rare bone tumor.