CADASIL: A NOTCH3-associated cerebral small vessel disease

•Progress of CADASIL like clinical, pathological, genetic, and therapeutic aspects is presented.•The role of NOTCH3 mutations in CADASIL is highlighted in this review.•Genetic testing is very important for diagnosis of CADASIL and CADASIL-like phenotypes.•The viewpoint CADASIL should be revisited as a NOTCH3-associated CSVD was proposed.•Hereditary CSVD caused by NOTCH3 mutations may be better understood with advanced research.