We are reporting the successful surgical treatment of a 23-year-old female with a giant right coronary artery to coronary sinus fistula. This woman had complaints of chest pain and dyspnea on exertion for few months. Transthoracic echocardiography (TTE) showed a large tortuous right coronary artery and a dilated coronary sinus. Preoperative multi-detector computed tomography (MDCT) coronary angiography and cardiac catheterization confirmed the diagnosis of a right coronary artery to coronary sinus fistula. The patient underwent surgical closure of the fistula and division of the communication between the right coronary artery and the coronary sinus with the use of cardiopulmonary bypass. The patient was discharged home on postoperative day 5 and at one-year follow-up is symptom-free.