Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) is an inherited small artery disease of mid-adulthood caused by mutations of the NOCTH3 gene on chromosome 19. Ischemic manifestations are the most frequent clinical events in CADASIL: 60-85% of patients have had transient ischemic attacks (TIAs) or complete strokes. Magnetic resonance imaging (MRI) shows on T2-weighted images widespread areas of increased signal in the white matter associated with focal hyperintensities in the basal ganglia, thalamus, and brainstem. Diagnostic testing with immunostaining using anti-NOCTH3 antibodies is an alternative method that seems easier than electronmicroscopy and particularly useful before initiating a complete screening of the gene in difficult cases. As CADASIL is a vascular disorder responsible for cerebral ischemic events, different authors prescribe aspirin for secondary prevention, but its benefit in the disease has not been shown. Treatment of migraine should be restricted to analgesic agents and nonsteroidal anti-inflammatory drugs.