Objective:o report a case of perivascular epithelioid cell tumor (PEComa) of the liver,and improve the understanding of the tumor. Methods:he clinical,imaging and pathological features of one case of liver perivascular epithelioid cell tumor admitted in October 2007 in Beijing Friendship Hospital Affiliated to Capital University of Medical Scienc was analyzed retrospectively and related literatures were reviewed. Results:he PEComa of liver didn't have any symptoms and characterized imaging features,and was difficult to make correct diagnosis before operation.Histologically,the resected tumor was composed of epithelioid cells,without vascular or lipomatous component,and was characterized by HMB45 positive on immunohistochemistry.Among present literatures,less than 10 cases of PEComa of the liver were reported. Conclusion:EComa is a very rare tumor of the liver which arises from mesenchymal tissues.The diagnosis of PEComa mainly depends on the pathological features.In respect of uncertain biologic potential of PEComa,long term follow up is indicated.