The normal balance between promotion and inhibition of calcification becomes dysregulated in chronic kidney disease, diabetes mellitus, atherosclerosis, and as a consequence of aging. Once the physiological determinants of calcification are perturbed, calcification may occur at several sites in the cardiovascular system, including the intima and media of vessels and cardiac valves. Clinically, 4 different types of cardiovascular calcification exist: atherosclerotic calcification, medial artery calcification, cardiac valve calcification, and vascular calciphylaxis. These 4 types are the consequence of distinct yet overlapping pathological mechanisms, and they are by no means mutually exclusive of one another. In particular, medial and atherosclerotic calcification occur frequently in concert and contribute synergistically to disease. Atherosclerotic calcification occurs at sites of atherosclerotic plaques, where there is a combination of cellular necrosis, inflammation, and cholesterol deposition. In contrast to atherosclerotic calcification, medial artery calcification (MEC) proceeds through a process similar to matrix vesicle-mediated intramembranous bone formation, with no cartilage intermediate required. Cardiac valve calcification is a more amorphous, disorganized process with aspects similar to both medial and atherosclerotic calcification. Finally, vascular calciphylaxis or calcific uremic arteriolopathy is a more systemic process characterized by diffuse calcification of the media of small to medium sized arteries and arterioles with intimal proliferation resulting in tissue necrosis.