As the most prevalent valvular heart disease, calcific aortic valve disease (CAVD) is a major health problem with risk of severe morbidity and mortality in the absence of effective medical treatment beyond surgical or interventional aortic valve replacement. The pathology involved in CAVD is multifactorial, including valvular endothelial cells damage, valvular interstitial cells differentiation, extracellular matrix remodeling, inflammation, fibrosis and calcification. Various risk factors for CAVD have been reported, such as age, gender, smoking, hyperlipidemia, hypertension, obesity and bicuspid aortic valves. Recently, diabetes mellitus has also been shown to accelerate the progression of CAVD. CAVD patients complicated with diabetes mellitus may benefit from early aortic valve replacement when compared with those without diabetes mellitus. Hence, diabetes mellitus is considered as an independent risk factor for CAVD. Therefore, in-depth understanding of the pathogenesis of these two diseases and their relationship may help us find appropriate prevention and therapeutic strategies for CAVD patients complicated with diabetes mellitus.