Objective To explore the CT and pathological features of renal epithelioid angiomyolipoma (EAML). Methods Clinical data and CT images from ten cases with EAML proved by surgery and pathology were retrospectively analyzed. All cases were performed with plain and contrast enhanced CT scans. Results CT features: higher pre-contrasted density than kidney, bulging from kidney,absent of fat, markedly heterogeneous enhancement ( quick wash-in and slow wash-out), big size without lobular sign, complete capsule with clear margin and mild necrostic area. Pathological features: diffuse sheets of epithelioid cells were found under microscopy with immunohistochemistrical findings including positivity for HMB-45 and negativity for EMA. Conclusion Some specific CT features, which is correlated well with the pathological findings, provide helpful information in the primary diagnosis of EAML.