Vertebrobasilar dolichoectasia (VBD) is characterized by ectasia, elongation, and tortuosity of the vertebrobasilar arteries, with a high degree of variability in clinical presentation. The disease origin is believed to involve degeneration of the internal elastic lamina, thinning of the media secondary to reticular fiber deficiency, and smooth muscle atrophy. The prevalence of VBD is variable, ranging from 0.05% to 18%. Most patients with VBD are asymptomatic and their VBD is detected incidentally; however, it is important to recognize that the presence of symptoms, which can lead to clinically significant morbidity and sometimes mortality, may influence clinical management. The most important clinical presentations of VBD are vascular events, such as ischemic stroke and catastrophic intracranial hemorrhage, or progressive compressive symptoms related to compression of adjacent structures, including the cranial nerves, brainstem, or third ventricle, causing hydrocephalus. The imaging diagnostic criteria for computed tomography and magnetic resonance (MR) imaging include three quantitative measures of basilar artery morphology: laterality score, height of bifurcation, and basilar artery diameter. The authors review the relevant anatomy and disease origin of VBD; pertinent imaging findings, including intraluminal thrombus and relation to the cranial nerves; and imaging pitfalls, such as the hyperintense vessel sign on MR images and artifacts related to slow flow in the dolichoectatic vessel. In addition, clinical manifestations, the role of radiology in diagnosis and management of this condition, and available management options are reviewed. (©)RSNA, 2016.