We present a case of Caroli's disease, a relatively uncommon congenital abnormality, together with CT, US, and scintigraphic findings. The patient had chronic renal failure, complicated by polycystic kidney. No hepatic fibrosis was observed. Intrahepatic biliary lithiasis and gallstones were detected, but the patient was asymptomatic. Because surgery on an abnormal biliary tract may increase the risk of cholangitis, and intravenous administration of contrast media could be invasive in patients with polycystic kidney, CT, US, and scintigraphy are considered to play an important role in diagnosis. Noninvasive diagnostic procedures in Caroli's disease are described in this report.