The right aortic arch with coarctation of the aorta was reported. A 56-year-old woman admitted to the hospital because of headache and hypertension. Cardiac catheterization revealed the right aortic arch with coarctation of the aorta and 80 mmHg pressure gradient across the coarctation. The bypass operation with a 14 mm Dacron graft between the ascending to descending aorta was performed. There was no peak systolic pressure gradient between the ascending and descending aorta after bypass operation. This patient is the fourth case report with both mirror-image type right aortic arch and coarctation of the aorta.