Approximately 60 to 85% of patients with CADASIL will experience recurrent ischemic events, which are primarily characterized by unilateral lacunar infarctions, Clinical manifestations that are characteristic of acute bilateral subcortical infarctions are very rare in CADASIL.Here, we report a case with an acute bilateral subcortical infarction as the primary clinical manifestation and carrying a rare mutation of the NOTCH3 gene. Here, NOTCH3 is linked to CADASIL.