Malakoplakia is an uncommon chronic inflammatory disease that appears as soft plaques in various organs and results from defective macrophage function, which tends to affect immunocompromised and debilitated patients. The pseudotumoral form presentation is rare especially with para-neoplastic syndrome. Preoperative diagnosis of renal malakoplakia in appropriate clinical settings can prevent unnecessary surgery. We present a clinical case of renal malakoplakia in a 58 old woman mimicking a malignant locally advanced renal carcinoma with rare presentation of bilateral pulmonary embolism.