Cervical carotid artery (cCA) dolichoectasia (DE) is characterized by elongation, tortuosity, and/or dilatation. The prevalence of cCA DE has been reported 13-31% in population-based and 14-58% in hospital-based studies. The exact mechanisms of this aberrant arterial remodeling are unknown. Although atherosclerosis has often been implicated, the evidence has conflicting results that would support atherosclerosis as the underlying pathology. Actually, other nonatherosclerotic mechanisms related to connective tissue remodeling may play a role. Such mechanism is supported by epidemiological evidence that cCA DE is associated with carotid dissections. Similarly, cCA DE has been associated with vascular risk factors, but inconsistently. Fewer studies have evaluated the risk of vascular events in people with cCA DE. Cross-sectionally, cCA DE is associated with cerebrovascular disease, including white matter hyperintensities, lacunar stroke, and stroke overall. The often-conflicting results may in part be due to the heterogeneity of the population studies and variable definitions used. Preferential use of objective measure of cCA DE, such as carotid length, is advisable, and may help comparing result among different studies. Prospectively, people with cCA DE have a higher risk of vascular events, although it is uncertain if the risk of stroke is also higher in this population. In the absence of alternative stroke etiologies, stroke patients with cCA DE should be considered to have had a cryptogenic stroke and treated with daily antiplatelet therapy. Further population-based studies are needed to clarify whether specific therapies may be implement to reduce the risk of events among people with cCA DE.