Acute type A aortic dissection (ATAAD) continues to be a subject of active research due to its high mortality rates and associated complications. Cerebral malperfusion in ATAAD can have a devastating impact on patients' neurological function and overall quality of life. We aimed to explore the risk profile and prognosis in ATAAD patients presenting with preoperative imaging signs of cerebral malperfusion (PSCM). We obtained patient data from our Aortic Dissection Registry, which included 480 consecutive ATAAD cases who underwent surgical repair between 2001 and 2021. Primary endpoint outcomes included the in-hospital and 30-day mortality, postoperative new neurological deficit, mechanical ventilation hours, and intensive care unit (ICU) length of stay. Of the total cohort, 82 patients (17.1%) had PSCM. Both groups had similar distributions in terms of age, sex, and body mass index. The patients in the PSCM group presented with a higher logistic EuroSCORE (47, IQR [31, 64] vs. 24, IQR [15, 39]; p < 0.001) and a higher portion of patients with a previous cardiac surgery (7.3% vs. 2.0%; p = 0.020). Intraoperatively, the bypass, cardioplegia, and aortic cross-clamp times were similar between both groups. However, the patients in the PSCM group received significantly more intraoperative packed red blood cells, fresh frozen plasma, and platelets transfusions (p < 0.05). Following the surgery, the patients who presented with PSCM had markedly longer ventilation hours (108.5 h, IQR [44, 277] vs. 43 h, IQR [16, 158], p < 0.001) and a significantly longer ICU length of stay (7 days, IQR [4, 13] vs. 5 days, IQR [2, 11]; p = 0.013). Additionally, the patients with PSCM had significantly higher rates of postoperative new neurological deficits (35.4% vs. 19.4%; p = 0.002). In the Cox regression analysis, PSCM was associated with significantly poorer long-term survival (hazard ratio (HR) 1.75, 95%CI [1.20-2.53], p = 0.003). Surprisingly, hypertension was shown as a protective factor against long-term mortality (HR: 0.59, 95%CI [0.43-0.82], p = 0.001). PSCM in ATAAD patients is linked to worse postoperative outcomes and poorer long-term survival, emphasizing the need for early recognition and tailored management.