Background: The optimal perfusion strategy during surgery of acute type A aortic dissection is controversial. The purpose of this study was to determine the impact of retrograde cerebral perfusion during hypothermic circulatory arrest on short-term and long-term outcome in this specific patient population. Methods: Between 1984 and 2005, 175 consecutive patients underwent repair of an acute type A dissection. Three different surgical approaches were used: aortic cross-clamping without hypothermic circulatory arrest in 50 (29%), hypothermic circulatory arrest alone in 69 (39%), and hypothermic circulatory arrest with supplemental retrograde cerebral perfusion in 56 (32%). Results: Operative mortality was 18% ± 3% (± 70% confidence interval), and adverse outcomes (death or cerebrovascular accident) occurred in 21% ± 3% of patients (p = 0.97 between groups). Multivariate analysis identified valve replacement (p = 0.04), preoperative flow complications (p = 0.03), and non-Marfan syndrome (p = 0.04) as predictors of operative mortality. Intraoperative dissection (p < 0.001) and history of cerebrovascular disease (p = 0.02) were predictors for permanent neurologic deficit, and retrograde cerebral perfusion was shown to have a protective effect on transient neurologic deficits (p = 0.008). Kaplan-Meier survival was 75% ± 3% at 1 year (131 patients at risk), 63% ± 4% at 5 years (87 patients at risk), and 49% ± 4% at 10 years (48 patients at risk) and was independent of surgical approach (p = 0.37). Long-term survival was diminished with increased age (p < 0.001), earlier operative year (p < 0.001), and coronary artery disease (p = 0.02). Conclusions: The current investigation suggests improved neurologic recovery with circulatory arrest and supplemental retrograde cerebral perfusion. Operative mortality and long-term survival were comparable among groups.