Epithelioid angiomyolipoma (eAMLoma) is an uncommon renal mesenchymal tumor with malignant potential. It is composed of tumor cells arranged in an epithelioid manner. Differential diagnosis from renal cell carcinoma is often challenging because of its epithelioid morphology. Herein is reported a case of eAMLoma, involving a 49-year-old man with eAMLoma. The patient had undergone radical nephrectomy via retroperitoneal laparoscope successfully. He had an uneventful postoperative recovery. The tumor was positive for Desmin, Hmb45, and Sma. We recommend surgical treatment and a follow-up regimen similar to that for renal carcinoma. There was no recurrence and metastases after 1-year follow-up.