Objective To explore the clinical features, diagnosis and treatment of renal epithelioid angiomyolipoma(EAML). Methods 7 cases of renal epithelioed angiomyolipoma(EAML)diagnosed in our unit from December 2012 to March 2014 were reviewed on their clinical and histopathological feathers retrospectively. There were 2 males and 5 females with a mean age of 43(26-69) years. 6 cases were detected in original examination without symptoms and one was admitted for hematuria. None of them had the history of nodular sclerosis. Result All the cases were treated by surgery. 6 cases were diagnosed as renal carcinoma preoperatively and one of them was accompanied by several enlarged lymph nodes in the retroperitoneal region and multiple pulmonarySmetastasis. 4 of the cases were underwent renal radical nephrectomy and 3 were underwent nephron sparing surgery. Immunohistochemcial staining of these tumors showed positive HMB45 and Melan-A but negative CK and EMA which supported EAML. There was no developments in the follow-up of 15.1 months (ranging 9-24 months) in all cases. Conclusions Renal EMAL was a rarely malignant potentiality neoplasm and it is necessary to identify preoperatively. Close follow-up is needed.