A 18 year-old female was admitted to our hospital with hypertension of 190/100 mmHg in her right arm and 140/110 mmHg in the left arm. Femoral arterial pulsation was not palpable. Her physical status and growth of the lower limbs were normal. Aortography demonstrated slight coarctation with a pressure gradient of 10 mmHg and descending aortic stenosis with a pressure gradient of 80 mmHg. Main three branches from aortic arch were anatomically normal. However, the left subclavian artery distal to the origin of the left vertebral artery was not visualized and the axillary artery was perfused by collaterals. Atypical coarctation was replaced using a woven Dacron graft of 14 x 40 mm. Postoperatively proximal pressure decreased from 180/113 to 156/98 mmHg and there was no pressure gradient between proximal and distal of the graft. After six months blood pressure in the right arm gradually decreased to 120/78 mmHg.