Epithelioid angiomyolipoma of renal origin are a very rare variant of the classic angiomyolipomas but with the peculiarity to present malignant potential. Its diagnosis is usually after surgery because the tumor simulates both clinical and radiological features of renal cell carcinoma. We report a case of a 14 year old patient who was diagnosed with this disease as an incidental finding, and that after she underwent partial nephrectomy for three years is currently asymptomatic with no signs of disease recurrence.