Background: Organ malperfusion is a serious complication of acute type A aortic dissection. Management and outcome of malperfusion has been discussed in this study. Methods: Between November 1994 and May 2003, 118 patients with acute type A aortic dissections were operated. Fifty-seven patients (48.3%) were complicated with organ malperfusion, which is considered as group I. Seventy-three ischemic events were seen in 57 patients with organ malperfusion. Patients in group I were divided into four subgroups according to affected organ system including limb (38 events), coronary (9 events), renal (2 events), visceral (9 events), and cerebral (15 events) ischemia. Sixty-one patients without organ malperfusion constitute group II. Results: The hospital mortality rate was 42.1% (24 of 57) in patients with malperfusion, 14.75% (9 of 61) in group II (p = 0.001), and 27.9% (33 of 118) in all patients. Postoperative complications such as mediastinal hemorrhage, low cardiac output, gastrointestinal system complications, acute renal failure, and multiple organ failure were higher in group I. Mesenteric and limb ischemia associated with high mortality. Multivariate analysis reveals that visceral malperfusion is the strongest predictor of postoperative mortality (odds ratio: 25.09, p = 0.000). Isolated coronary malperfusion had the lowest mortality (one patient, 16.6%) among the patients with organ malperfusion. Conclusions: Acute type A aortic dissections with organ malperfusion has higher postoperative mortality and morbidity. Immediate aortic repair is our management strategy in patients with limb, coronary, and neurological malperfusion. To reduce the extremely high mortality with mesenteric malperfusion, new strategies should be investigated such as surgical delay with interventional procedures.