目的　结合病史观察血管平滑肌脂肪瘤 (angiomyolipoma ,AML)的病理组织学特点及免疫组化表达特征。方法　光镜观察结合免疫组化及组织化学法。结果　AML由血管、平滑肌、脂肪三种成份构成 ,部分细胞可有异型性 ,偶可见核分裂。免疫组化染色 :Actin(+)、Vimentin(+)、HMB45 (+)、EMA(- )。结论　以上对肉瘤或肾癌鉴别诊断有较大帮助。细胞的异型性不足以作为诊断恶性肿瘤的标准 ,HMB45阳性在诊断和鉴别诊断中有一定价值 Objective To observe the histopathological features and immunohistochemical characteristics of angiomyolipoma (AML) combined with medical history. Methods Light microscopy combined with immunohistochemistry and histochemistry. Results AML by blood vessels, smooth muscle, fat three components, some cells may have atypia, even see the nuclear fission. Immunohistochemical staining: Actin (+), Vimentin (+), HMB45 (+), EMA (-). Conclusion The above diagnosis of sarcoma or kidney cancer have a greater help. Cell atypia is not enough to diagnose malignant tumors, HMB45 positive in the diagnosis and differential diagnosis of a certain value