Mitral annular calcification (MAC) is a chronic, degenerative process of the fibrous annulus of the mitral valve. MAC occurs in 8–15% of elderly patients and is usually an incidental finding on echocardiography or chest CT. As the population ages and life expectancy increases, severe mitral valve disease (stenosis and regurgitation) combined with severe MAC will be an increasing challenge requiring optimal treatment. Today, transcatheter mitral valve replacement (TMVR) is steadily gaining ground and is applied with high success rates in high surgical risk patients. However, proper preoperative evaluation of candidates for each procedure has significantly improved the prognosis of these patients by reducing complication rates and overall mortality.