Acute type A aortic dissection and coarctation of the aorta is a rare associated disease. A case of two-stage repair is presented. Firstly the ascending aorta and the right hemi-arch was replaced using deep hypo-thermic circulatory arrest. Cardiopulmonary bypass was proximally instituted, in a patient with total aortic isthmus occlusion, using right axillary artery cannulation. Distally arterial perfusion was obtained cannulating the bilateral hypoplasic femoral arteries. Ten months later a left subclavian artery-descending thoracic aorta bypass was performed.