Background: We assessed the clinical and computed tomographic (CT) features of localized cystic disease of the kidney and how these features differentiate this disease from other renal cystic diseases. Methods: Medical records and CT scans of seven patients with localized cystic disease were reviewed retrospectively. Pathologic confirmation in five patients was done by surgery. The subjects consisted of four males and three females, with an age range of 29–74 years. Results: Localized cystic disease in five patients was an incidental finding. Clinical presentations in the other two patients included dark urine color, palpable flank mass, and generalized weakness. There was no patient with impairment of renal function associated with renal cystic disease. Six patients had cystic renal lesions in the left kidneys, and one patient had lesions in the right kidney. Six patients had cysts in a localized area in the affected kidney, and the remaining patient had diffuse involvement of the entire kidney. The upper pole was most frequently involved. CT appearance was characterized by multiple, variable size cysts conglomerated to islets of cysts that were separated by the normal renal parenchymal band. Conclusion: Localized cystic disease of the kidney has characteristic CT findings that distinguish it from other renal cystic diseases, making follow-up without surgical intervention possible.