The cellular and molecular basis of congenital heart disease (CHD) is an evolving area of rapid discovery. This article introduced the basic mechanisms underlying cardiac development and CHD in order to permit a clear understanding of current diagnostics and therapeutics and their future development. It is clear that although significant advances have been made in understanding mechanisms controlling heart formation, the direct causes of CHD remain poorly defined. Future studies tha delineate the complexity of these mechanisms are required to provide a comprehensive understanding of the etiologies of CHD. Such understanding will lead to the development of novel approaches to prevention and therapy.