Malakoplakia involving the genitourinary tract is a rare inflammatory disorder that presents a diagnostic challenge. Renal parenchymal involvement is particularly uncommon. We report a case of bilateral renal malakoplakia that presented with acute renal failure and simulated xanthogranulomatous pyelonephritis (XGP). The etiology, clinical course, and management of malakoplakia are reviewed, emphasizing the distinct characteristics of the disease that lead to its accurate diagnosis.

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