Arrhythmogenic Right Ventricular Dysplasia/Cardiomyopathy (ARVD/C) is an inherited cardiomyopathy characterized clinically by ventricular arrhythmias, sudden death, and structural abnormalities of the right ventricle. The genetics of ARVD/C has provided support for the hypothesis that ARVD/C is a disease of desmosomal dysfunction. Diagnosis of ARVD/C relies upon a scoring system with major and minor criteria based on the demonstration of a combination of defects in RV morphology and function, characteristic depolarization/repolarization electrocardiogram (ECG) abnormalities, characteristic tissue pathology, ventricular arrhythmias, family history, and the results of genetic testing. Establishing an accurate diagnosis, risk stratification and deciding when to implant an implantable cardioverter defibrillator, minimizing symptoms and prevent implantable cardioverter-defibrillator therapies, and prevent progression are the four major components of management of ARVD/C. The management of patients with ARVD/C is primarily aimed at reducing the burden of symptomatic arrhythmias and decreasing the incidence of sudden cardiac death. An important consideration in patients with ARVD/C is preventing disease progression, and this can be accomplished by exercise restriction.