This review summarizes the pathophysiology of mitral annular calcification (MAC) with recent findings and current strategies for diagnosis and treatment. Major factors in MAC development seem to be shear stress of the flow past the mitral valve, local inflammation, and dysregulation in regulators of mineral metabolism. MAC itself poses daunting technical challenges. Implanting a valve on top of the calcium bar might lead to paravalvular leak (PVL) that is less likely to heal. Annular decalcification allows for better valve seating and potentially better healing and less PVL. This, however, comes with the risk for catastrophic atrioventricular groove disruption. MAC can be sharply dissected with the scalpel; the annulus can be reconstructed with the autologous pericardium. Transcatheter mitral valve replacement is a promising approach in the treatment of patients who are deemed high-risk surgical candidates with severe MAC. MAC is a multifactorial disease that has some commonalities with atherosclerosis, mainly regarding lipid accumulation and calcium deposition. It is of great clinical importance, being a risk marker of cardiovascular events (including sudden death) and, with its progression, can have a negative impact on patients' lives.