Abstract: Ischemic stroke (IS) is one of the leading causes of mortality and disability in the world. In recent years, elevated plasma homocysteine has been studied for a potential role in ischemic stroke. Methylenetetrahydrofolate reductase (MTHFR) is a key enzyme in homocysteine metabolism, and its changes of enzyme activity, can affect the level of plasma homocysteine. Recently, the relationship between the gene polymorphism of MTHFR and IS has gradually been a hot research. This article discusses the international advances in this field.