目的结合近期收治的1例肝脏血管周上皮样细胞肿瘤（perivascular epitheliod cell tumor，PEComa）的病例特点，总结该疾病的诊治体会。 方法回顾分析2013年9月笔者所在医院成功收治的1例肝脏原发PEComa的诊治过程，并结合既往文献报道，总结和复习该疾病的临床特点以及处理方法。 结果肝脏PEComa较为罕见，起病隐匿，症状缺乏特异性，临床诊断困难，多依赖病理学检查证实，手术切除是有效的治疗手段。 结论提高本病影像学特征的认识有助于术前肿瘤性质的判断，本病预后较好，但术后仍需要长期密切观察随访。
Perivascular epithelioid cell tumor (PEComa) is a multi-potential tumor based on mesenchymal cells distributed around capillaries. The main affected population is female, and the clinical manifestations are not specific. It can affect all parts of the body. There are more PEComa in the uterus and very few PEComa in the liver. Due to its low incidence, clinicians lack awareness of it. Based on the relevant literature, this article reviews the clinicopathological features, imaging features, molecular phenotypes, diagnosis, differential diagnosis, and treatment of liver PEComa, so as to strengthen the understanding of the disease, prevent missed diagnosis and misdiagnosis, and guide clinical work.