Mitral annular disjunction (MAD) is an underdiagnosed structural abnormality of the mitral valve apparatus in which the mitral leaflet hinge point is towards the atrium. MAD is associated with mitral valve prolapse and, importantly, increased rates of ventricular arrhythmias, and sudden cardiac death. In this case report and review, we describe the case of a 37-year-old man incidentally found to have MAD during a workup for paroxysmal atrial fibrillation and review the existing literature on the epidemiology, diagnostic methods, natural history, and clinical implications of MAD. Our goal is to highlight important gaps in current guidelines regarding the optimal treatment of patients with MAD.