Chronic kidney disease (CKD) is an independent risk factor for cardiovascular diseases, including stroke. Patients with CKD are susceptible to ischemic as well as hemorrhagic stroke. The impairments in the small vessel vasculature, atherosclerotic changes of the large vessels, and coagulation abnormalities in CKD probably underlie the specific characteristics of stroke in these patients. The clinical outcomes, including the functional outcomes and short- as well as long-term mortality after stroke, are poor in patients with CKD. CKD is defined as a decreased glomerular filtration rate (GFR) and/or increased urine albumin excretion. A number of studies have shown that either or both of these two markers were significantly associated with poor functional outcomes and mortality after stroke. Recent studies have suggested that proteinuria/albuminuria is more deeply involved in the clinical outcomes than GFR. Although the mechanisms responsible for their association are currently unclear, glomerular barrier and/or filtration dysfunction are probably related to the small vessel diseases, hypercoagulability and inflammation, which may affect the clinical outcomes in CKD patients after stroke. The evidence for the most effective management of acute stroke in CKD patients is lacking, and thus, the current treatment for stroke is optimized for individual patients based on their background. Further studies are thus needed to elucidate the specific features of stroke and also the management of stroke in patients with CKD.