The current internationally recognized etiology of congenital heart disease is mainly the result of a combination of heredity and the environment factors. Environment-induced changes in epigenetic level may be a key reason for the origin of the disease, which have important clinical significance, and DNA methylation is a major epigenetic mechanism. This article reviews the progress on some environmental factors and DNA methylation and their association with congenital heart disease so that we can better understand the pathogenesis of congenital heart diseases, which is good for early diagnosis and early treatment.