Objective To explore the pathological and imaging features of epithelioid angiomyolipoma(EAML). Methods A 47-year-old man presented with dull pain in left upper abdomen for 1 year admitted to hospital.Accompanied with several enlarged lymph nodes,a mass with the largest diameter of 28 cm in the left kidney was inhomogeneously enhanced on CT.The left kidney and the mass were radically resected with regional lymph node dissection after general anaesthesia. Results During the operation,a well-circumscribed,encapsulated mass with several paraaortic lymph nodes was found on the upper-middle left kidney.The gross cut surface showed fleshy texture with regions of necrosis.Microscopically and immunohistochemistrically,diffuse sheets of epithelioid cells were found with positivity for HMB-45 and negativity for EMA.CT features included higher pre-contrasted density,absent fat,enhanced mode of quick wash-in and slow wash-out.There was neither metastasis nor recurrence during the 1 year follow-up. Conclusion EAML with malignant potency is a rare tumor of mesenchymal tissue presenting with some specific radiopathological features and a good prognosis.