Objective To evaluate the imaging features and diagnosis of 64-Multidetector coronary CT angiography(64-MDCTA) on congenital coronary artery fistula(CAF). Methods A total of 12 patients with CAF were given 64-MDCTA scan, acquired satisfactory image, did the post-processing for volume rending(VR), multiplanar reconstruction(MPR) and maximum intensity projection(MIP), and compared with the results of coronary angiography(CAG). Results 64-MDCTA results showed that, a total of 22 fistulas were found in 12 patients with CAF, single fistula was 41.7%(5/12), multiple fistulas was 58.3%(7/12), combined with fistula aneurysm was 25.0%(3/12). 64- MDCTA detected 1 fistula origin of the left subclavian artery(LSCA), and 64-MDCTA and CAG were identical in the diagnosis of the remaining 21 fistulas. The missed diagnosis rate of VR diagnosis fistula entrance was 9.1%(2/22), MPR was 18.2%(4/22) and MIP was 54.5%(12/22). The missed diagnosis rate of VR and MPR significantly less than MIP(χ2 =10.476, 6.286, P=0.001, 0.012). The missed diagnosis rate of VR and MPR were no statistic difference(χ2 =0.772, P=0.380). The missed diagnosis rate of VR diagnosis fistula exit was 13.6%（3/22), MPR was 18.2%(4/22) and MIP was 59.1%(13/22). The missed diagnosis rate of VR and MPR significantly less than MIP(χ2 =9.821, 7.765, P=0.002, 0.005). The missed diagnosis rate of VR and MPR had no statistic difference(χ2 =0.170, P=0.680). Conclusion 64-MDCTA has good value in the diagnosis of CAF.