We conducted a systematic review of published studies on stroke epidemiology in American Indians and Alaska Natives (AI/ANs). We used MeSH terms and strict inclusion criteria to search PubMed, identifying a relevant sample of 57 refereed publications. We report a consensus view in which prevalent stroke is more common, and estimates of cerebrovascular risk factors are higher, among AI/ANs than among other US populations. Like other minority groups, AI/ANs suffer stroke at younger ages than do non-Hispanic Whites. However, data on AI/AN stroke mortality are significantly compromised by racial misclassification and nonrepresentative sampling. Studies correcting for these problems have found that stroke mortality rates among AI/ANs are among the highest of all US racial and ethnic groups. As with Black and non-Hispanic White stroke mortality, AI/AN stroke mortality varies by geographic region, with the highest rates in Alaska and the Northwest and the lowest in the Southwest. Our results underscore the need for a concerted national effort to collect accurate cross-sectional and longitudinal data on stroke in AI/ANs.