Abstract Arrhythmogenic right ventricular dysplasia (ARVD) is an inherited cardiomyopathy and is also called ARVD/C. In most cases, ARVD is inherited in an autosomal-dominant pattern and clinically is characterized by ventricular arrhythmias with an increased risk of sudden death. Although it is prominently a disease of the right ventricle (RV), involvement of the left ventricle is not uncommon. Pathologically, there is RV myocardial atrophy with thinning of the wall, aneurysm, and global RV dilatation. Genetic testing and magnetic resonance imaging of the RV are an increasing aspect of the diagnosis. Diagnosis relies on a scoring system with major or minor criteria. Prevention of sudden death is the primary goal of management. Pharmacologic treatment of arrhythmias, catheter ablation of ventricular tachycardia, and implantable cardioverter defibrillator are the mainstay of treatment of ARVD/C.