plenic tumors are more rarely seen comparing to other visceral organ tumors. Primary tumors of spleen are divided into non-lymphoid and lymphoid groups. Vascular neoplasms of non-lymphoid tumors consist the vast majority part. In the literature review, among splenic hemangioendothelioma cases, rarely of them were reported as primary splenic epithelioid hemangioendothelioma. Its frequency among splenic tumors is 0.3-14%. These tumors do not become in large sizes and have better prognosis. If they become large, they may cause symptoms such as pancytopenia and hemolytic anemia due to splenomegaly and hypersplenism. Early diagnosis and treatment may prevent conditions such as widespread metastasis, splenic rupture and operative complications. In this article, we present clinical and imaging findings of epitheloid splenic hemangioendothelioma with review of other reported cases in the light of literature.