The malperfusion syndrome associated with acute aortic dissection draws attention because the clinical picture is very dramatic, and patients with malperfusion have poorer clinical outcomes. To improve surgical outcomes, the ischemic damages associated with organ malperfusion should be minimized by restoring perfusion as early as possible, which occasionally can hardly coexist with central repair. This paper reviews the current evidence, problems, and dilemma related to the diagnosis and treatment of the malperfusion syndrome caused by acute type A aortic dissection.