The sporadic form of the disease shows a solitary lesion often connected to a developmental venous anomaly, while the familial form harbors multiple lesions throughout the brain and has been associated to an autosomal dominant mutation in one of the three CCM genes (CCM1/KRIT1, CCM2/OSM or CCM3/PDCD10) [8, 39, 62]. Here, KRIT1 is linked to Cerebral venous angioma.