Homocysteine is a sulfur-containing amino acid needed by human body and an important intermediate metabolite of methionine cycle. In recent years, hyperhomocysteinemia as an independent risk factor of cardiovascular disease has attracted wide attention. At present, more and more evidence shows that hyperhomocysteinemia plays an important role in the occurrence and development of arrhythmia. The purpose of this review is to elucidate the relationship between hyperhomocysteinemia and arrhythmia, and to find new ways to prevent arrhythmia and reduce its mortality.