Epithelioid hemangioendothelioma (EHE) is a rare vascular tumor with histological features between hemangiomas and high-grade angiosarcomas. Because its imaging and histological features are similar to hemangiomas or metastatic spinal tumors, accurate diagnosis and effective treatment of spinal EHE are particularly important. From some of the reported spinal EHE, we can note that there are certain similarities in its diagnosis and treatment, and this article reports one case of spinal EHE, and reviews and summarizes the published literature on spinal EHE.