目的　提高肾血管平滑肌脂肪瘤的诊断与治疗水平。方法　总结分析 36例肾血管平滑肌脂肪瘤病人的临床资料。结果　B超诊断准确率 77 7% (2 8/ 36 ) ,CT诊断准确率 83 3% (30 / 36 ) ,治疗以保留肾单位的手术为主 ,对于病变小无症状者采用定期观察 ,病变大者 ,则采取肿瘤切除、肾部分切除或肾切除术。结论　应提高对肾血管脂肪瘤的术前诊断水平 ,采取各种措施保留肾单位。 Objective To improve the diagnosis and treatment of renal angiomyolipoma. Methods The clinical data of 36 patients with renal angiomyolipoma were analyzed. Results The diagnostic accuracy rate of B ultrasound was 77 7% (28/36), and the diagnostic accuracy rate of CT was 83 3% (30/36). The operation was mainly based on the surgery of retaining nephron, and the regular observation of lesion asymptomatic The larger, then take the tumor resection, partial nephrectomy or nephrectomy. Conclusions The level of preoperative diagnosis of renal vascular lipoma should be increased and various measures should be taken to keep nephron.