Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) is originally featured with a strong clustering of mutations in NOTCH3 exons 3–6 and leukoencephalopathy with frequent anterior temporal pole involvement. The gene discussed is NOTCH3; the disease is cerebral arteriopathy with subcortical infarcts and leukoencephalopathy.