目的:分析肝脏血管周上皮样细胞瘤的临床特点。方法:2007年10月首都医科大学附属北京友谊医院普外科诊治1例肝脏血管周上皮样细胞瘤，对其临床、影像、病理学特点进行分析及相关文献复习。结果:肝脏血管周上皮样细胞瘤无临床症状，无特征性影像学表现，术前难于确诊。组织学上由单一上皮样细胞构成，不含血管及脂肪成分，免疫组化呈特征性黑色素生成（HMB-45）阳性。目前文献报道肝脏血管周上皮样细胞瘤发生在肝脏者尚不足10例。结论:肝脏血管周上皮样细胞瘤是一种非常罕见的间叶组织来源肿瘤。主要依靠病理进行诊断。肝脏血管周上皮样细胞瘤生物学行为不确定，建议术后长期随访。

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.