Vertebral and basilar arteries are the major blood suppliers of the brain. They supply nearly one-third of the brain. Congenital absence of these arteries is a rare condition. Usually, this condition presents with stroke like presentation. This is a case presentation of a 40-year-old lady who was diagnosed as congenital agenesis of vertebra-basilar arteries. This lady was presented with intraventricular hemorrhage. Subsequently the diagnosis was made by cerebral angiogram.