We aimed to undertake a meta-analysis of cohort studies to evaluate gender-based differences for patients with acute type A aortic dissection. A systematic search was performed across PubMed, Embase, and Cochrane Library (2000 to 2023) for studies reporting gender-related discrepancies in clinical presentation, in-hospital management, and/or outcomes. Study effects were assessed using mean difference or risk ratio (RR) as aggregated estimates. Besides, individual patient-level data on survival were reestablished to form gender-related Kaplan–Meier curves to evaluate long-term survival outcome. The study protocol was registered in PROSPERO (ID: CRD42024524125). The 21 studies were analyzed, comprising 6,728 women and 12,839 men. Women had lower risks of postoperative acute kidney injury (RR 0.85; 95% confidence interval [CI] 0.72 to 1.00, p = 0.049) and reoperation (RR 0.89; 95% CI 0.81 to 0.99, p = 0.024) but a higher perioperative mortality (RR 1.11; 95% CI 1.03 to 1.18, p = 0.005) than men. In addition, the overall survival was poorer in women (p <0.001), with 10-year survival rates of 66.5% for men and 60.0% for women. In conclusion, acute type A aortic dissection presents gender differences, with women facing higher perioperative and long-term mortality despite lower acute kidney injury and reoperation risks, suggesting a need for tailored management and prevention strategies.