A 10-year-old boy was admitted to our hospital with severe hypertension due to unusual aortic coarctation at the level of the diaphragm without renal artery stenosis. We made left interior thoracotomy and left para-rectal incision through extraperitoneal approach, and extra-anatomic bypass was established with a 16 mm knitted Dacron graft from the descending aorta to the infrarenal abdominal aorta, under a circulatory assist. Postoperatively, he complained of abdominal pain with residual hypertension and required an intensive anti-hypertensive treatment to avoid intestinal necrosis. Pressure gradient between upper and lower extremities disappeared 3 weeks after repair. High level of plasma renin activity still continued 4.5 months after surgery in spite of oral administration of beta blockade and ACE inhibitor.