We report a rare case of type B aortic dissection associated with coarctation of the aorta. A 35-year-old man had sudden dyspnea and severe back pain. Computed tomography revealed aortic coarctation at the distal aortic arch, and aortic dissection below the coarctation. The diameter of proximal descending aorta was enlarged to 52 mm. We electively performed excision of aortic coarctation and descending aortic graft replacement. Coarctation of the aorta has a poor prognosis, The risk of aortic rupture due to aortic dissection is very high, and the histological abnormality is also pointed out. Therefore we should perform aggressive surgical treatment.