Malakoplakia is an uncommon chronic granulomatous inflammatory disease which is associated with immunocompromised conditions such as malignancy, autoimmune disease, chronic alcohol intake, poorly controlled diabetes and long-term steroid use. Malakoplakia can occur at various sites, most commonly in the genitourinary tract including urinary bladder and the ureter. Renal parenchymal involvement is relatively uncommon, accounting for 15% of all malakoplakia. A few cases of renal malakoplakia have been reported in Korea, and only one case was accompanied by acute kidney injury. Here we report an 80-year-old female patient with renal parenchymal malakoplakia and acute interstitial nephritis presented as acute kidney injury with literature review.