Abstract: Objective:To analyze the clinicopathological characteristics, diagnosis, and differential diagnosis of hepatic perivascu-lar epithelioid cell neoplasm (PEComa). Methods:The clinicopathological features of seven hepatic PEComa patients treated in Shanxi Cancer Hospital from March 2008to March 2015were studied with light microscopy and immunohistochemistry combined with litera-ture review. Results:The age range of the seven patients was41- 56years old (median: 51.5 years old). Two were males and five were females. Their histopathological subtypes were classic type ( 2/7), smooth muscle type ( 3/7), and fat type (2/7), respectively. Immunohis -tochemical study showed that HMB 45was positive in7 cases, SMA and VIM were expressed in6 cases, Melan A was positive in 5 cas-es, and Desmin was expressed in 2 cases. In addition, the mean proliferative index was 1.3% (range: 0%-5%), and TFE3 was negative in all cases. Conclusion:Hepatic PEComa is a rare primary liver tumor. The diagnosis and treatment of this disease should be intensi-fied, and long-term close follow-up is necessary. Positive result for HMB45, Melan A, and SMA are the key features of PEComa.