OBJECTIVE. This study was undertaken to evaluate color Doppler imaging findings in patients with Budd-Chiari syndrome and to compare these findings with results of venography. SUBJECTS AND METHODS. In a prospective study, 21 patients with proved Budd-Chiari syndrome had color Doppler imaging. Sonographic evaluations were performed to detect appropriately directed flow in the hepatic veins, portal vein, and inferior vena cava. Intrahepatic collaterals were characterized when present. Results of color Doppler imaging were compared with those of angiography in 20 patients. Color Doppler images of the hepatic veins were also obtained in a reference group (20 control subjects, 20 patients with hepatomegaly, and 20 patients with cirrhosis). RESULTS. Color Doppler imaging showed abnormalities of anatomy or flow in one or more of the main hepatic veins in all 21 patients with Budd-Chiari syndrome. Commonly observed abnormalities were visualization of a hepatic vein on real-time sonograms that had no flow or retrograde flow on color Doppler sonograms (11 cases) and no visualization of part or all of a hepatic vein on either real-time or color Doppler sonograms (10 cases). When compared with venographic findings (16 patients), findings on color Doppler sonograms could be used to distinguish patent from occluded hepatic veins in all cases. In our reference group, real-time and color Doppler sonograms showed normal hepatic veins in all control subjects. Real-time sonograms clearly showed hepatic veins in 12 of 20 patients with hepatomegaly; color Doppler sonograms showed flow in the hepatic veins in all 20 of these patients. Among 20 patients with cirrhosis, real-time sonograms showed hepatic veins in only seven; color Doppler imaging confirmed patent veins in 17. Intrahepatic collaterals typical of Budd-Chiari syndrome were observed in 10 of 21 patients with the syndrome. The portal vein was assessed by using color Doppler imaging in all 21 patients with Budd-Chiari syndrome; portograms were available for comparison in 10 patients. Findings were consistent in eight; in two cases, the direction of flow was reversed on color Doppler sonograms compared with portograms. For the inferior vena cava, venographic and sonographic findings correlated in 16 of 20 cases. Color Doppler sonograms did not show a caval web in one patient. CONCLUSION. Abnormalities of the hepatic veins, portal veins, and inferior vena cava detected on color Doppler sonograms in patients with Budd-Chiari syndrome correlate well with findings on venograms. The presence of intrahepatic collateral vessels may be important in distinguishing patients with Budd-Chiari syndrome from those who have cirrhosis or inconspicuous hepatic veins.