Supravalvular aortic stenosis is an uncommon congenital narrowing of the ascending aorta that may be localized or diffuse, originating at the superior margin of the sinuses of Valsalva just above the level of the coronary arteries. The most common complication of supravalvular aortic stenosis is early onset of intimal hyperplasia and atherosclerosis of the coronary arteries. The coronary arterial lesions of supravalvular aortic stenosis are dilatation or coronary artery ostial obstruction. We experienced a case of supravalvular aortic stenosis combined with right coronary artery ostial obstruction. A 21 year-old female patient was admitted because of exertional dyspnea and chest pain for 2 months. Cardiac catheterization showed a narrowing of ascending aorta with prominent calcification in the lesion and moderate aortic valve insufficiency. The peak to peak left ventricular-supravalvular aortic pressure gradient was 54 mmHg. Selective coronary angiography revealed as a complete obstruction of the ostium of the right coronary artery. Surgical correction was performed successfully. Postoperative left ventricular-supravalvular aortic pressure gradient was decreased to 22 mmHg. Postoperative clinical course was favorable and she was discharged with good condition. We present a case of supravalvular aortic stenosis combined with right coronary artery ostial obstruction with a review of literatures.