Angiomyolipoma of the kidney is a common benign mesenchymal neoplasm of kidney. A rare variant, epithelioid angiomyolipoma, however, may show malignant behavior. We report a case of epithelioid angiomyolipoma in a patient not having tuberous sclerosis which was initially misdiagnosed as renal cell carcinoma. A 39-year-old woman presented with a history of flank pain. Ultrasonography revealed a left renal mass. Contrast-enhanced computed tomography (CECT) abdomen revealed mass involving hilum of the kidney. On core biopsy a possibility of renal cell carcinoma was suggested. The patient underwent radical nephrectomy. After immunohistochemical analysis, a final diagnosis of epithelioid angiomyolipoma was made. Renal epithelioid angiomyolipoma without adipocytic component is extremely rare. It is pivotal to keep a possibility of epithelioid angiomyolipoma whenever an epithelioid renal tumor is encountered showing marked pleomorphism and mitosis. The use of melanocytic markers and specific markers of renal cell carcinoma will aid the diagnosis.