Objectives: To assess sex differences of clinical presentation and outcomes in propensity-matched patients with acute type A aortic dissection (AAAD). Methods: We collected the clinical data of patients with AAAD from a single heart center between January 2009 and July 2014. After propensity score matching, we compared differences in clinical presentation and outcomes of patients with AAAD between men and women. Results: There were 590 patients (295 men and 295 women) with AAAD through propensity matching on demographics and patients' history. We found that the presentation and diagnosis of AAAD often were more delayed in women. Severe signs of congestive heart failure (9.8% vs. 5.1%, P = 0.017), cardiac tamponade/shock (9.1% vs. 4.1%, P < 0.001), and periaortic hematoma (26.4% vs. 21.7%, P < 0.001) were more commonly presented in women. Surgery was more commonly performed in men than in women (95.4% (281/295) vs. 91.5% (270/295), P = 0.045), indicating the association of sex with surgical decision. To investigate the association of sex with outcomes after surgery, patients who underwent surgical treatment were re-matched (262 men and 262 women) by propensity score. Women suffered from greater in-hospital mortality than men (8.4% vs. 3.4%, P < 0.001). Postoperative complications of congestive heart failure (9.1% vs. 3.8%, P < 0.001), visceral ischemia (6.8% vs. 1.1%, P < 0.001), and limb ischemia (7.6% vs. 1.5%, P < 0.001) were more frequent in women. For women, prolonged operative time may increase in-hospital mortality, especially after 12 hours from the start of surgery (30.0% vs. 14.3%, P < 0.001). Kaplan-Meier survival analysis indicated worse late outcomes in women in the matched surgery group (log-rank P = 0.012). Conclusions: Our analysis provides new insights into sex differences in clinical presentation and outcomes of AAAD.