Malakoplakia is an uncommon but distinctive type of chronic granulomatous inflammation that occurs most commonly in the genitourinary tract, especially the urinary bladder. Most patients have associated conditions characterized by some degree of immunosuppression, as seen in solid-organ transplants, autoimmune diseases requiring steroid use, chemotherapy, chronic systemic diseases, alcohol abuse and poorly controlled diabetes. We report an unusual case of the renal malakoplakia that involved the perirenal space, extending to the descending colon in a 65-year-old Korean woman with secondary adrenal insufficiency and diabetes mellitus.