To evaluate the clinical, radiological, and genetic features of cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) with intracerebral hemorrhage (ICH) involvement. Methods: Three patients admitted to our hospital with CADASIL who presented with ICH symptoms, and additional 25 cases in the Pubmed database using the MeSH terms CADASIL, intracerebral hemorrhage, leukoencephalopathy, and NOTCH 3 were enrolled. Clinical, radiological, and genetic features of the 28 cases were analyzed. Results: Of the 28 patients, 9 were female and 19 male, and the mean age was (54.2 ± 13.4) years. Hypertension was the most common risk factor and was present in 18 patients (64.3% ). Fifty percent of patients received antithrombotics to prevent ischemic events. ICH was the initial manifestation in 10 patients, and the most frequent sites of ICH were the basal ganglia and brain lobes. R544C in exon11 was the most prevalent mutation (46.2%). Cerebral microbleeds varying in number were found in 18 patients (64.3% ). Conclusion: ICH may present in CADASIL patients and may be the initial clinical symptom. In addition, strict control of hypertension and cautious use of antithrombotics may be important in the prevention of ICH in CADASIL patients.