Our objective was to describe the main aspects of MR imaging in Caroli's disease. Magnetic resonance cholangiography with a dynamic contrast-enhanced study was performed in nine patients with Caroli's disease. Bile duct abnormalities, lithiasis, dot signs, hepatic enhancement, renal abnormalities, and evidence of portal hypertension were evaluated. Three MR imaging patterns of Caroli's disease were found. In all but two patients, MR imaging findings were sufficient to confirm the diagnosis. Moreover, MR imaging provided information about the severity, location, and extent of liver involvement. This information was useful in planning the best therapeutic strategy. Magnetic resonance cholangiography with a dynamic contrast-enhanced study is a good screening tool for Caroli's disease. Direct cholangiography should be reserved for confirming doubtful cases.