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Localized renal cystic disease (LRCD) is a rare benign non-hereditary, non-progressive condition which must be differentiated from other renal cystic diseases. Familiarity with this condition and its characteristic imaging features avoids unnecessary surgical intervention. We report a case of LRCD in a 40-year-old male who presented with left loin pain. Computed tomography demonstrated a cluster of simple cysts in the lower pole of the left kidney with normal right kidney and other organs. Diagnosis of LRCD was made on typical imaging characteristics and absence of family history of cystic renal disease.