Abstract: A case of hepatic epithelioid hemangioendothelioma with ¹⁸F-FDG PET/CT whole-body imaging was reported. The characteristics of the disease were analyzed from clinical symptoms, laboratory and pathological examination, and imaging, and were understood through literature review. The disease has an extremely low incidence and the clinical manifestations are lack of specificity. It is not easy to be found in the early stage. It is difficult to be distinguished from common liver tumors, and easy to be misdiagnosed. The prognosis of the disease is poor. It is recognized at present that the most effective treatment is liver transplantation or radical resection.