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。 Objective To summarize the experience of diagnosis and treatment of one case of perivascular epitheliod cell tumor (PEComa) treated recently. Methods The diagnosis and treatment of one case of primary liver PEComa successfully treated in our hospital at September 2013 was retrospectively analyzed. The clinical features and treatment of the disease were summarized and reviewed according to previous reports. Results Liver PEComa was rare, occult onset, lack of specificity of symptoms, clinical diagnosis difficult, and more dependent on pathological examination confirmed that surgical resection is an effective treatment. Conclusion To improve the understanding of the imaging features of the disease contribute to the judgment of the nature of the tumor before surgery, the prognosis of the disease is better, but still need long-term follow-up after surgery.