Herein we review 100 cases of adult polycystic kidney disease. Flank pain was the most common presentation in these cases. In 20% of the patients calculi were noted and 10% had obstruction. The average patient age at death was 57 years. The importance of tomography is emphasized with key radiographic features, such as bilateral large kidneys, inhomogeneous nephrogram, caliceal distortion and poorly defined renal margins. Cystic involvement of the liver and spleen may help to confirm the diagnosis. Differential diagnosis includes bilateral multiple simple cysts, angiomyolipomatosis and bilateral renal carcinomas.