Mitral annular disjunction is a cardiac structural abnormality characterized by the distinct separation between the top of the left ventricular myocardium and the mitral annulus supporting the posterior mitral leaflet occurring during systole. It has recently gained wide attention due to the increasing recognition of the link between mitral annular disjunction and arrhythmogenic mitral valve prolapse, particularly, with the increased risks of ventricular arrhythmias resulting in sudden cardiac death. This review has summarized the recent progress in the diagnostic modalities, clinical implications of mitral annular disjunction, and its specific surgical considerations.