A giant coronary artery aneurysm (GCAA) concurrent with coronary artery fistula is a rare condition, and it becomes even more unusual when combined with a single coronary artery (SCA) anomaly. Here we report such an extremely rare case, who is a 35-year-old woman presenting with severe chest distress. A GCAA with fistula to the right ventricle was noted, occurring in a single coronary artery, diagnosed by multimodality cardiovascular imaging techniques. Both GCAA and coronary artery fistula can cause severe cardiac complications, which jeopardize life. While an SCA is mostly asymptomatic, it may also lead to sudden cardiac death as well. Therefore, surgical intervention was recommended. We chose a novel thrombus-inducing strategy to eliminate the GCAA and repair the fistula. Symptoms were relieved after the surgery, and the patient remained asymptomatic over 8 months of follow-up.