It is well known that cerebrovascular disease has become an important cause of adult death and disability. Strikingly, the Tibet Autonomous Region (TAR) ranks on the top in China for the incidence of stroke. To help explain this phenomenon, we have searched for and analyzed stroke-related literature for the TAR in the past 2 decades and have referenced reports from other regions at similar altitudes. This article focuses on epidemiology features, risk factors, and pathogenesis of stroke in the TAR in an effort to generate a better understanding of the characteristics of stroke in this region. The special plateau-related factors such as its high elevation, limited oxygen, the high incidence of hypertension, smoking, and the unique dietary habits of the region are correlated with the high incidence of stroke. In addition to these factors, the pathogenesis of stroke in this high-altitude area is also unique. However, there is no established explanation for the unique occurrence and high incidence of stroke in the TAR. Our study provides an important rationale not only for the clinic to prevent and treat this disease, but also for the government to develop appropriate health policies for the prevention of stroke in the TAR.