Liver is one of the most common organs to be affected by epithelioid hemangioendotheliomas (EHs) that are called primary hepatic EHs (HEHs). Due to the rarity of the disease, different aspects of these malignant tumors remain unclear, including their etiopathogenesis. No specific and characteristic clinical manifestations have been reported for HEH since almost 25% of patients with HEH are asymptomatic at the time of diagnosis. Thus, the majority of the tumors are detected incidentally by diagnostic imaging carried out for other reasons. Computed tomography (CT) and magnetic resonance imaging (MRI) are widely used diagnostic approaches for the diagnosis of HEH, considering some characteristics and distinguishing features of the tumor in these approaches. Angiosarcoma, cholangiocarcinoma, hepatocellular carcinoma, metastatic carcinoma, and sclerosing hemangioma can be considered the main differential diagnoses for HEH, which should be differentiated via imaging and histological evaluations. Surgical treatment, within the frame of liver resection and liver transplantation (LT), is currently the only curative option for HEH that can increase long-term survival. Therefore, liver resection should be considered the gold standard for all patients with HEH, except those with unresectable disease. In these cases, liver transplantation should be considered the last resort for curative treatment, although systemic disease may prevent some patients from being eligible for transplantation.