Introduction: Acute aortic dissection is the most common cause of death in patients with Marfan syndrome and untreated aortic root enlargement. Emergency surgery for replacement of the ascending aorta has the potential of life saving procedure, but is associated with high morbidity and mortality. Long-term outcomes after surgical repair of acute aortic dissection type A in patients with Marfan syndrome are limited. Material and methods: We made a retrospective study concerning emergency surgical intervention for acute aortic dissection type A, by Bentall procedure, performed in Emergency Institute for Cardiovascular Diseases „Prof dr. C. C. Iliescu” Bucharest between January 2005 and July 2014. We included 332 patients with type a acute aortic dissection divided into two groups: group A - 16 patients with Marfan syndrome and group B - 316 patients with other etiologies. We analyzed differences between these two groups regarding perioperative characteristics, surgical technique and short and long-term morbidity and mortality. Results: The patients from group A were significantly younger than those in group B (35.1±12.7 years vs. 56.8±7.1 years; p<0.001). Arterial hypertension was three times more common in group B as compared to group A (p<0.001). The incidence of postoperative morbidity, and intraoperative and 30-days mortality death rates were similar between groups, but overall mortality at 10 years was lower in group A (31.3%) vs. group B (44.9 %). Conclusions: Emergency surgical in Marfan syndrome, by Bentall procedure could improve perioperative morbidity and mortality of patients with acute aortic dissection, but need an early diagnosis, proper medical therapy and imagistic surveillance.