Mood disorders, cognitive decline, headaches, and stroke are among the key symptoms of cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy, or CADASIL [1]. Rarely, ataxia has been noted as a presenting ailment in this case [2]. A man 48 years of age showed signs of developing ataxia. Magnetic resonance imaging (MRI) of the brain showed focal tiny infarct in lest internal capsule, subacute intraparenchymal hemorrhage involving lest temporal lobe, few old lacunal infarct in bilateral ganglio capsular region, right thalamus and bilateral fronto-parietal periventricular white matter. A mutation in the NOTCH3 gene was verified through genetic testing. It found a variant c.268C>T (p.Arg90Cys). We also address the potential etiology of cerebellar ataxia linked to CADASIL and other factors that could lead to a different differential diagnosis. CADASIL is an uncommon cause of progressive ataxia.