Dilated cardiomyopathy(DCM) is a myocardial disorder characterized by cardiac dilatation and contractile dysfunction of the left and/or right ventricles,which is one of the cause of heart failure in addition to coronary heart disease and hypertention.Familial DCM may account for 35% of DCM. The mutations of sarcomere filament protein and cytoskeletal protein were largely found in familial DCM,and the mutations in mitochondrial DNA were also found in familial DCM.In this review,we focus on the molecular genetics of familial DCM.