Cardiolipin (CL), one of bisphosphatidyl glycerol lipid, is found to contain high level of C18:2 acyl chains and exist exclusively in inner membranes of mitochondria. Cardiolipin remodeling is the process for CL to attain its specific acyl chains. With the purpose of introducing this interesting field to readers, in this review, we elucidate the role of cardiolipin in normal functions of mitochondria by analyzing the physical and chemical characters of cardiolipin; we introduce the current models of cardiolipin remodeling by analyzing the enzymes involved in this process; we reviewed the latest progress in the clinical implications of cardiolipin remodeling.