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Mitral annular calcification (MAC) is a progressive, degenerative process increasingly recognised for its clinical impact. Beyond being an incidental finding, MAC contributes to mitral valve dysfunction, arrhythmias, systemic embolisation and elevated cardiovascular risk. In developed countries, it has now overtaken rheumatic disease as the leading cause of mitral stenosis.The pathophysiology of MAC involves chronic mechanical stress, pro-inflammatory activation and osteogenic differentiation of valvular cells. Progression is accelerated by age, chronic kidney disease and metabolic derangements. Diagnosing MAC-related valve dysfunction is challenging, as traditional echocardiographic measures often prove unreliable. Multimodality imaging—including 3D echocardiography and cardiac CT—is essential for assessing anatomy, function and procedural feasibility. Importantly, symptoms often reflect combined valvular (eg, aortic stenosis) and myocardial disease (eg, heart failure with preserved ejection fraction (HFpEF) phenocopy), necessitating careful haemodynamic evaluation to avoid futile interventions.Management should prioritise medical therapy for symptom control and comorbid HFpEF, reserving interventions for selected patients. Surgical and transcatheter approaches carry high risk and should be undertaken only in specialised centres. Future advances may include tailored devices and therapies targeting calcification pathways.