The clinical course of a 60-year-old gentleman with a history of atypical 
migraine, recurrent encephalopathic episodes and progressive cognitive 
impairment is presented. He was diagnosed with cerebral autosomal dominant 
arteriopathy with subcortical infarcts and leucoencephalopathy, a rare genetic 
disorder of the cerebral blood vessels caused by mutations in the Notch 3 gene 
on chromosome 19. The diagnosis was confirmed by MRI, skin biopsy and genetic 
testing. His cognitive function has progressively deteriorated and he continues 
to receive supportive care provision. The course and review of the condition are 
highlighted.