Objective: Renal angiomyolipoma is a mesenchymal neoplasm composed of dysmorphic blood vessels, smooth muscle, and adipose tissue. Epithelioidangiomyolipoma is an uncommon variant considered potentially malignant. We report a case of renal epithelioid angiomyolipoma suspicious for urothelialcarcinoma of renal pelvis.

Methods: A 50-year-old woman with a 3-day history of macroscopic hematuria and CT-scan showing a tumor arising from the left renal pelvis suspicious for urothelialcarcinoma. Nephrectomy was analysed in multiple slides of hematoxilin-eosin and immunohistochemistry.

Results: Left nephrectomy was performed. Tumor sized 8 cm and showed pathologic findings of epithelioid angiomyolipoma with a little component of classic angiomyolipoma. We studied differential diagnosis and pathologic characteristics for the prognosis.

Conclusions: Pathologic study in detail is fundamental for differential diagnosis and prognosis of this tumor. Our patient's pathology showed good prognosis, however follow-up is important due the malignant potential of the neoplasm.

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