Epithelioid angiomyolipoma (EAML) is a rare subtype of angiomyolipoma (AML) with potential has malign tendency. UP to now, the biological association between EMAL and typical AML is not well known. In the present study, we reported a case of EMAL arose in AML seven years post AML operation. A 34-year-old man with bilateral kidney AML was operated with an open cytoreductive surgery of both sides. After seven years, the left nephrectomy was performed due to a new neoplasm was found in the left kidney. The pathology diagnosis and inmmunohistochemical staining suggested the new neoplasm was EAML. Finally the patient developed metastases to liver and retroperitoneum and died 19 months post the last operation. After reviewing the history of the patient, we thought that the EAML might be developed from a little amount of EMAL accompany with AML in the small lesson that was missed by the CT scan. The results revealed that a carefully screening for the epithelioid cells in the typical AML specimen should be made to avoid missing the important EAML.