Clinical, radiographic, and surgical findings in a case of multilocular renal cyst are presented. The history, physical examination, and laboratory findings, including excretory urogram, renal scan, nephrotomogram, and arteriogram were compatible with the diagnosis of renal cell carcinoma. The urologist's awareness of this entity may prevent erroneous treatment in children. In adults, it may allow the preservation of uninvolved renal parenchyma, particularly in a solitary kidney.
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