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The authors retrospectively reviewed a consecutive series of patients with diffuse intracranial dolichoectasia and compared demographics, vascular risk factors, additional aneurysm prevalence, and clinical outcomes with a group of patients with vertebrobasilar dolichoectasia. Twenty-five patients had diffuse intracranial dolichoectasia, and 139 had vertebrobasilar dolichoectasia. Patients with diffuse intracranial dolichoectasia were older than those with vertebrobasilar dolichoectasia and had a higher prevalence of abdominal aortic aneurysms, other visceral aneurysms, and smoking history. Patients with diffuse intracranial dolichoectasia were more likely to have aneurysm growth. They conclude that the natural history of patients with diffuse intracranial dolichoectasia is significantly worse than that in those with isolated vertebrobasilar dolichoectasia.