The vertebral arteries constitute the proximal segment of the cerebral posterior circulation and are often divided into two segments (extra- or intracranial) based on their relationship with the dural space. Diseases affecting these arteries can be asymptomatic or can lead to a variety of well-described stroke syndromes, such as the lateral medullary syndrome. Atherosclerotic plaque formation with subsequent occlusion is the most common stroke mechanism, although atheroembolic phenomena from proximal vessels or the heart and arterial dissection with distal embolization are frequent. Diagnosis is mainly through noninvasive studies such as brain computed tomography (CT) or magnetic resonance imaging (MRI) or CT angiography/MR angiography, but cerebral angiography remains the gold standard for the evaluation of cerebral arteries. Treatment varies according to the portion of the vessel affected but is mainly focused on maximizing cerebrovascular risk factors and the initiation of antiplatelet therapy. Prospective randomized trials studying the different treatment modalities for individuals with vertebral artery disease are needed to improve our understanding of this potentially devastating entity.