Abstract: Objective: To summarize the imaging characteristics of autosomal dominant hereditary cerebral arteriopathy with subcortical infarct and white matter encephalopathy (CADASIL), and to discuss the value in the view of the STandards for ReportIng Vascular changes on nEuroimaging（STRIVE）. Methods: A total of 7 cases of 3 families and some family members with NOTCH3 mutant gene were studied. The clinical data were collected and the MRI scan of the head were performed ,including SWI sequences of some patients. 4 of the patients were followed up for 2-4 years.Results: 6 cases of the 7 patients had early onset of adulthood. The main clinical manifestations included dizziness, migraine, progressive dementia, recurre nt ischemic stroke, and 1 case was completely asymptomatic. 7 cases of head MRI all showed multiple lacunar foci of bilateral cerebral hemisphere, mainly distributed in the basal ganglia, thalamus, pontine, corpus callosum and semi oval centers. 7 cases all had white matter high signal, of which 4 cases were distributed in the bilateral anterior temporal lobe and 6 cases were in the external capsules; 5 cases had the subcortical small infarct, mainly distributing in the basal ganglia, paraventricular, semi oval centers, 2 cases of cerebral microbleeds in basal ganglia, 1 case showed brain atrophy.Conclusion: The manifestations of MR could explain the characteristics of the imaging markers of s mall cerebral vascular disease. The high signal intensities on T2W and FLAIR sequences of bilateral anterior temporal lobe and external capsule is of high diagnostic value for the diagnosis. The change of white matter high signal intensities distribution can reflect the worsing of the disease to a certain extent. Although perivascular space and cerebral atrophy are characteristic of cerebral small vessel disease, the practical value in the diagnosis of CASDSIL is limited