A rare case of neonatal congenital coronary artery, right ventricle fistula with giant coronary artery aneurysm formation, was reported. Computed tomography angiography demonstrated the dilated and tortuous tunnel arising from the right aortic sinus and traversing the epicardial surface before opening into the anterolateral aspect of the RV. Successful surgical repair was performed with a patch closure of the fistula and coronary angioplasty. The postoperative recovery was uneventful. Our experience of this rare congenital heart disease demonstrated that early surgical repair of coronary artery fistula and coronary angioplasty in the neonate can be performed safely. Further study is needed to seek the basis on this.