Chronic kidney disease (CKD) is an independent risk factor for cardiovascular disease and also increases the incidence of cerebrovascular disease. Prevention and management of cerebrovascular disease in patients with CKD, especially endstage kidney disease (ESKD), are different from those without CKD because of their specific pathophysiology. In patients with ESKD on hemodialysis, a decrease in brain blood flow due to a decline in blood pressure during hemodialysis might cause incident cerebrovascular disease. Cerebral microbleeds detected by magnetic resonance imaging, which are much more prevalent in patients with CKD compared with the general population, might also cause incident brain hemorrhage. For management for the hyperacute phase of brain infarction, thrombolytic therapy using recombinant tissue plasminogen activator has been recently indicated for those patients. However, this indication should be carefully evaluated because the risk of adverse events is higher in patients with CKD, especially ESKD. More recently, mechanical thrombectomy has also been applied clinically. However, the efficacy of this treatment remains unknown in CKD and ESKD patients. With regard to prevention, carotid endarterectomy and carotid artery stenting are performed for severe carotid artery stenosis. Similarly to the treatment described above, the indication for these procedures should be carefully evaluated because the incidence of stroke, myocardial infarction, and mortality is considered to be high in patients with CKD. Administration of warfarin to hemodialysis patients with atrial fibrillation is controversial because whether atrial fibrillation is involved in incident brain infarction and whether warfarin prevents cerebrovascular disease remain unknown in those patients. Novel oral anticoagulants are now available but are prohibited in severe CKD patients. Further investigation and accumulation of evidence are required for prevention and management of cerebrovascular disease in patients with CKD, especially ESKD.