Abstract: Mitochondria are considered to be the cellular power plants and pivotal signaling hubs. It is known that mitochondria plays an essential role in regulating cell proliferation, differentiation, and survival. A kinase anchoring protein 1 (AKAP1) functions as a scaffold protein that is involved in modulating mitochondrial functions and mitochondria-related physiological and pathophysiological processes through collecting protein kinase A (PKA) and other signaling proteins and RNAs to the outer mitochondrial membrane. It has been reported that AKAP1 is involved in cardiovascular physiology and pathophysiology, including myocardial hypertrophy, myocardial apoptosis and vasodilation. Herein, this review reveals that it is significant to appreciate and to understand current knowledge about AKAP1 and molecular mechanisms of AKAP1-related signaling complexes in the regulation of mitochondrial functions and cardiovascular diseases.