Abstract: Objective To study the clinical characteristics, diagnosis and differential diagnosis of hepatic epithelioid hemangioendothelioma(HEHE). Methods Clinical data and pathological characteristics of 6 patients with HEHE diagnosed by hepatic puncture were analyzed. Results Of the 6 HEHE patients, 2 were males and 4 were females at a mean age of 45 years (range:25~58 years). Their main clinical manifestations were right upper abdominal pain, fever, fatigue, and weight loss. Two patients were asymptomatic. Pathology and imaging showed multiple intra-hepatic metastases in 5 patients, single metastasis in 1 patient, and lung metastasis in 3 patients. Histology revealed short spindle cells characterized by dendritic and epithelioid cells, epithelial cells in cytoplasm, interstitial compact fibrosis and mucoid degeneration. Immunohistochemistry demonstrated positive CD34 in 6 patients, positive CD31 in 5 patients, positive vimentin in 5 patients, occasional positive CK and FVIIIRAg. Of the 3 patients who received hepatic embolism intervention, 1 underwent additional cold ablation and chemotherapy, 1 received additional microwave ablation therapy, 1 was going to undergo liver transplantation. The patients were followed up for 8 months~14 years, during which 5 were alive and 1 missed follow-up. Conclusion HEHE is a rare tumor. Its final diagnosis depends on pathological and immunohistochemical findings. It should be differentially diagnosed from metastatic adenocarcinoma and cholangiocarcinoma, and treated by surgical operation or intervention.