Objective To investigate the imaging features of Budd-Chiari syndrome (BCS) on 64 slice spiral computed tomography (64SCT) and the diagnostic value of 64SCT for BCS. Methods Twenty-nine patients diagnosed as BCS by 64SCT were retrospectively included into this study and all the patients were researched by digital substraction angiography (DSA). Two abdominal radiologists analyzed the CT imaging features of BCS, paying attention to the vascular lesion, the morphology abnormality of the liver and the degree of portal hypertension, with review of DSA findings. Results ①The accuracy of 64SCT for BCS was 93.1% (27/29), and there were 2 false positive cases and no false negative case. The accuracy of 64SCT for those patients with thrombosis of inferior vena cava (IVC) and (or) hepatic vein (HV) was high as compared to those with stenosis of IVC and (or) HV. ②The morphology abnormality of the liver included hepatomegaly (24 cases), low attenuation (27 cases) and inhomogeneous pattern of parenchymal contrast enhancement (5 patients in arterial phase and 19 patients in portal vein phase). ③The images of all the patients showed the features of portal hypertension. Conclusion The accuracy of 64SCT for BCS is satisfactory and the false negative is seldom. The 64SCT could accurately display the morphology abnormality of the liver and the compensatory circulation in BCS patients. For those patients with stenosis of IVC and (or) HV, however, the diagnostic power of 64SCT is limited.