Both mitral annular disjunction and coronary artery anomalies are associated with ventricular arrhythmia, though data on their co-occurrence are limited. We herein present two patients with anomalous aortic origin of a coronary artery and mitral annular disjunction who had ventricular arrhythmia. Mitral annular disjunction should be considered as a potential cause of ventricular arrhythmia when stress tests rule out ischaemia.