Abstract: 　Mitochondria are the energy factory of all the cells, the center of aerobic metabolism, and essential for the metabolism of cells. Mitochondrial cardiomyopathy refers to myocardial damage caused by mitochondrial dysfunction and is characterized by cardiac structural and (or) functional abnormalities. The typical clinical feature of mitochondrial cardiomyopathy is hypertrophic cardiomyopathy, dilated cardiomyopathy, arrhythmias, noncompaction of left ventricular and heart failure. This article focuses on the pathophysiology, clinical manifestations and possible treatments of mitochondrial cardiomyopathy.