Vertebro-basilar dolichoectasia (VBD) is a rare pathology of unknown aetiology. Its clinical presentation is wide and prognosis is generally poor with a high mortality rate. Cerebral magnetic resonance imaging is the gold standard for diagnosis. We report an unusual case of intracranial dolichoectasia. VBD was revealed during investigation of a patient with altered mental status. CT brain imaging demonstrated severe obstructive hydrocephalus secondary to compression of the third ventricle. Management is always challenging and depends on the location and the mode of presentation. Our patient died despite surgical management with placement of an external ventricular shunt