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Malakoplakia is a granulomatous tissue inflammation with a characteristic histological appearance, mainly affecting the urogenital system and morphologically reflecting a macrophage disease. If bladder involvement is the most common, renal involvement is very rare and may be responsible for a differential diagnosis problem with renal cell carcinoma.We present a clinical case of renal malacoplakia mimicking malignant renal cell carcinoma diagnosed after partial nephrectomy in a 58-year-old woman with no history of recurrent urinary infections.