目的探讨肝脏上皮样血管平滑肌脂肪痛的临床病理特征、诊断及鉴别诊断。方法采用HE切片和免疫组织化学SP法染色对1例肝脏上皮样血管平滑肌脂肪瘤进行研究,并复习文献。结果上皮样血管平滑肌脂肪瘤免疫组化表达HMB45、Melan-A、SMA阳性。结论上皮样血管平滑肌脂肪瘤是一种罕见的间叶性肿瘤,其组织形态变化较大,若发生在肝脏,更易误诊;此例组织学类型应进一步分类为上皮样细胞型的血肿型。临床上依靠病理并辅助免疫组化检查进行确诊。 Objective To investigate the clinicopathological features, diagnosis and differential diagnosis of hepatic epithelioid vascular smooth muscle fatty acid pain. Methods One case of hepatic epithelioid angiomyolipoma was studied by HE section and immunohistochemical staining. The literature was reviewed. Results Epithelioid angiomyolipoma expressed HMB45, Melan-A and SMA positively. Conclusions Epithelioid angiomyolipoma is a rare mesenchymal tumor with large histological changes. If it occurs in the liver, it is more likely to be misdiagnosed. In this case, the histological type should be further classified as epithelioid cell type hematoma. Clinically dependent on the pathology and assisted by immunohistochemistry to confirm.