Epithelioid hemangioendothelioma (EHE) is a rare vascular tumor. In this report, we describe the case of a 62-year-old man who presented with pain in the left clavicle and swelling of the left upper limb. Contrast-enhanced computed tomography revealed an intravascular tumor, which was completely resected surgically. Histopathological examination and immunohistochemical staining revealed that it was epithelioid hemangioendothelioma with occurrence in the left brachiocephalic vein. It has been 6 years since the surgery was performed, and no recurrence has been observed. Epithelioid hemangioendothelioma may recur or metastasize and therefore requires careful follow-up.