Abstract: Mitochondria, containing ~1 000 different proteins, are the source of energy for a cell and are one of the most complex subcellular organelles. The proteins forming the functional mitochondrial network play indispensable roles in energy production, fatty acid metabolism, cell signaling, cell apoptosis and autophagy. Mitochondrial dysfunction is closely related to the occurrence and progression of cardiovascular disease. In this article we focus on the cardiac mitochondrial proteome, with emphasis on the changes associated with cardiovascular disease.