Migraine, and specifically migraine with aura (MA), has been associated with increased risk of major cardiovascular disease (CVD). Numerous studies have indicated that patients with migraine have an increased risk of vascular disease. The association between migraine and ischemic stroke was the earliest to be recognize but migraine has been associated also with myocardial infarction, hemorrhagic stroke, retinal vasculopathy, incidental brain lesions and with peripheral artery disease. To better define the biological link between migraine and vascular events, several mechanisms have been proposed, but the precise mechanisms are currently unknown. In this review, we briefly summarize the evidence supporting a clinical association between migraine and the most common cardiovascular disease, exploring hypothesis that may explain the association and dealing with gender differences and pharmacological treatment issues. Understanding the mechanism of cardiovascular disease in migraineurs may be useful in determining effective strategies for cardiovascular prevention and, for acute preventing treatment of migraine. The link of cardiovascular disease and peripheral artery disease with migraine may be present, but it is difficult to separate out from common vascular risk factors often present at the same time such as smoking, diabetes mellitus and hypertension. The presence of coronary artery disease or peripheral vascular disease limits the use of certain acute and preventive migraine treatments. Any migraine treatment that decreases the width of a blood vessel cannot be used in those who have or might have cardiovascular disease. Future targeted research is warranted to identify preventive strategies to reduce the risk of future cardiovascular disease among patients with migraine. These results further add to the evidence that migraine should be considered an important risk marker for cardiovascular disease, at least in women.