Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) is a genetic syndrome that presents as a vasculopathy or dementia caused by a mutation in the NOTCH3 protein. Spontaneous intracerebral hemorrhage (ICH) has rarely been reported in association with CADASIL. A 68-year-old Korean woman was admitted to our hospital because of sudden onset of dysarthria, headache and left hemiplegia. She was diagnosed with CADASIL three years ago. She had been taking cilostazol 100 mg daily for a year because of dizziness and gait ataxia. Brain CT revealed hyperacute intracerebral hemorrhage in the right basal ganglia with spot sign. Cilostazol may trigger the development of ICH in CADASIL. Careful risk-benefit assessment is required before prescribing antiplatelet agents for patients with CADASIL.