Vertebralbasilar dolichoectasia (VBD) is a relatively rare condition affecting mainly the tunica media of the vertebrabasilar arteries with poor prognosis. As is characterized by elongation and distension of arteries, it commonly shows excessively enlarged vessel diameter with weakened vessel walls and is accompanied by, sometimes, thrombus. Patients with VBD may suffer from catastrophic consequences caused by acute cerebral ischemia, acute rupture of the vascular, or progressive compression of the cranial nerves, the brainstem or the third ventricle. In this review, the author gives an overview of the pathology, pathophysiology and clinical manifestation of VBD, in hope to provide clinicians with a better understanding of the disease.