Objective: The use of the frozen elephant trunk (FET) technique for repair of type A aortic dissection (TAAD) in Marfan syndrome (MFS) is controversial. We seek to evaluate the efficacy of FETand total arch replacement (TAR) for TAAD in patients with MFS. Methods: The early and long-term outcomes were analyzed for 106 patients with MFS (mean age, 34.5 +/- 9.7 years) undergoing FET + TAR for TAAD. Results: Operative mortality was 6.6% (7 of 106). Spinal cord injury and stroke occurred in 1 patient each (0.9%), and reexploration for bleeding occurred in 6 patients (5.7%). Extra-anatomic bypass was the sole risk factor for operative mortality and morbidity (odds ratio [OR], 7.120; 95% confidence interval [CI], 1.018-49.790; P = .048). Follow-up was complete in 97.0%(96 of 99), averaging 6.3 +/- 2.8 years. Late death occurred in 17 patients. Patients with acute TAAD were less prone to late death than those with chronic TAAD (OR, 0.112; 95% CI, 0.021-0.587; P = .048). Twelve patients required late reoperation, including thoracoabdominal aortic repair in 8, thoracic endovascular aortic repair for distal new entry in 3, and coronary anastomotic repair in 1. At 5 years, survival was 86.6% (95% CI, 77.9%-92.0%) and freedom from reoperation was 88.8% (95% CI, 80.1%-93.4%), and at 8 years, survival was 74.1% (95% CI, 61.9%-83.0%) and freedom from reoperation was 84.2% (95% CI, 72.4%-91.2%). In competing risks analysis, mortality was 4% at 5 years, 18% at 8 years, and 25% at 10 years; the respective rates of reoperation were 10%, 15%, and 15%; and the respective rates of survival without reoperation were 86%, 67%, and 60%. Survival was significantly higher in patients who underwent root procedures during FET + TAR (P = .047). Risk factors for reoperation were days from diagnosis to surgery (OR, 1.160; 95% CI, 1.043-1.289; P = .006) and Bentall procedure (OR, 12.012; 95% CI, 1.041-138.606; P = .046). Conclusions: The frozen elephant trunk and total arch replacement procedure can be safely performed for TAAD in MFS with low operative mortality, favorable long-term survival and freedom from reoperation. A concomitant Bentall procedure was predictive of better long-term survival and increased risk for late reoperation. These results argue favorably for the use of the FET + TAR technique in the management of TAAD in patients with MFS.