A successful single-stage correction of coarctation of the aorta with aortic regurgitation is reported. A 39-year-old man was referred for angina, ankle swelling, and numbness of lower extremities. Aortogram revealed severe aortic regurgitation and coarctation of the aorta with 2.8 mm minimum diameter. Aortic valve replacement was performed under separate perfusion of upper and lower body, after which the coarctectomy and graft interposition were done under the left heart bypass. The patient was discharged in good condition with minor medication of digitalis and anticoagulant agents. As far as the case is concerned, the left heart bypass during graft interposition has possible risk of cardiac arrest and usual cardiopulmonary bypass is hazardous when complicated by severe coarctation of the aorta. Surgical procedure and assist methods used in this case were quite useful for coarctation of the aorta with aortic regurgitation.