Abstract Background In acute aortic dissection (AAD) sex heterogeneity reports are not exhaustive and in part even conflicting. Aims To explore sex differences in clinical features, management, and outcomes among patients with type A AAD. Methods and results A systematic review and meta-analysis of the literature were conducted for studies (2004–2022) reporting type A AAD sex differences. Among the 1938 studies retrieved, 16 (16 069 patients, 7142 women, and 8927 men) fulfilled all eligibility criteria. Data were aggregated used the random-effects model as pooled risk ratio and mean difference. Due to information reported by considered manuscripts, analysis were performed only among surgically treated type A AAD patients. At the time of hospital presentation type A AAD women were older than men but had lower body mass index (BMI), body surface area (BSA), and creatinine plasma levels. Active smoking, bicuspid aortic valve, and previous cardiac surgery were less common in women while diabetes mellitus was more frequent. Furthermore, women experienced more frequently pericardial effusion/cardiac tamponade than men. Interestingly, in-hospital surgical mortality did not differ between sexes [risk ratio (RR), 1.02; 95% confidence interval (CI), 0.53–1.99; P = 0.95], whereas 5 (RR 0.94; 95% CI: 0.92–0.97; P < 0.001) and 10-year survival (RR 0.82; 95% CI: 0.74–0.92; P = 0.004) was higher among men. A descriptive analysis of in-hospital outcomes among medically treated type A AAD patients confirmed prohibitive high mortality for both sexes (men 58.6% vs. women 53.8%, P = 0.59). Conclusions A female sex phenotype appears to be evident in type A AAD implying the need for a personalized management patient approach along with tailored preventive strategies. PROSPERO registry ID CRD42022359072.