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Malperfusion syndrome (MPS) complicating acute type A aortic dissection (ATAAD) poses a continuing challenge and management dilemma for cardiovascular surgeons. MPS may involve any of the major arterial side branches resulting in myocardial, cerebral, spinal cord, visceral, and/or limb ischemia with varying frequency and severity. Despite the continuous improvement in diagnosis and management strategies for MPS with ATAAD, clinical outcomes remain poor and the optimal therapy is still debatable. The present review aimed to assess current evidence on ATAAD patients with MPS and how best to handle the challenge.