Multiple hepatic perivascular epithelioid cell neoplasm (MHPEComa) is a really rare type of neoplasm derived from mesenchymal tumors. Hepatic multiple epithelioid angiomyolipoma is extremely rare in clinical practice. Few cases of MHPEComa have been described in the literature to date. A retrospective analysis was performed to investigate the clinicopathological characteristics of 2 patients diagnosed with MHPEComa. Two female patients revealed three and two lesions in the liver respectively, then received surgical resection. Microscopically, detection of epithelioid or spindle-shaped cells with fisheye-like nuclei was suggestive of the disease, and analysis of biomarkers, tumor cells were markedly positive with the melanocytic differentiation marker, Melan-A and HMB45. The hepatic markers were negative. After a follow-up period of 48–84 months, no recurrence was observed. That is often misdiagnosed as hepatocellular carcinoma (HCC), hepatic hemangioma or other liver malignancies. Surgical resection is a definitive curative treatment of hepatic multiple angiomyolipoma when tumour grows or malignancy cannot be excluded.