ObjectiveTo summarize clinicopathologic and immunophenotypic features of hepatic epithelioid angiomyolipoma (HEAML) and to explore its diagnostic and differential diagnostic methods.MethodThe clinical and imaging manifestations, pathological morphology and immunohistochemical features of 5 patients with HEAML from August 2011 to December 2017 in this hospital were retrospectively analyzed.ResultsThere were 2 males and 3 females in the 5 patients with HEAML, aged 38–64 years with an average age of 50 years. There were 2 cases of the left lobe tumors and 3 cases of the right lobe tumors. Three cases were diagnosed as the hepatocellular carcinoma and the other two cases were diagnosed as the hepatic hamartoma and (or) hemangioma by the preoperative imaging examination. The diameter of tumors ranged from 1.5 cm to 7.0 cm, with an average of 3.6 cm. Microscopically, the tumors were composed of more epithelioid smooth muscle cells, parenchyma vessels and a small amount of fat. The immunohistochemical results showed that the melan-A, HMB45, and SMA were positive, while the HepPar-1, AE1/AE3, EMA, CD117, Dog-1, CD10, CgA, Syn, and Desmin were negative. The Ki-67 proliferation index was 2%–10%. The patients were all alive without the tumor recurrence after following up for 2–76 months with an average of 31.4 months.ConclusionsHEAML is a rare primary mesenchymal tumor of liver, which should be misdiagnosed for other benign or malignant tumors for influencing clinical treatment. Diagnosis and differential diagnosis can be made by histopathology and immunohistochemical staining.