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Epithelioid angiomyolipoma is a rare mesenchymal tumor arising mainly in the kidney that can potentially behave aggressively. Epithelioid angiomyolipoma can often resemble sarcomatoid renal cell carcinoma, high grade renal carcinoma or sarcoma. Its similarity to renal cell carcinoma has been emphasized in most of the cases reported in literature. With the purpose of contributing to the awareness of this similarity, a 32-year-old female patient with renal epitelioid angiomyolipoma in the left kidney which radiologically mimicked urothelial cell carcinoma of the upper urinary tract is presented.