The most common initial symptom of acute aortic dissection is chest or abdominal pain. Nevertheless, in a minority of cases, it may have an atypical presentation, making the diagnosis clinically challenging. This article reports on a case of acute type A aortic dissection presenting as mental status confusion. The diagnostic suspicion for a cerebrovascular accident may have catastrophic consequences in a clinical condition, which is associated with a high mortality during the first 48 hours after the onset of symptoms if untreated. The right diagnosis was made by combining a careful physical examination, echocardiography, and computed tomography. The available literature about neurologic manifestations, their pathophysiology and prevalence as the initial symptom of acute type A aortic dissection is reviewed.