Abstract Arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C) is an inherited cardiomyopathy that is characterized clinically by ventricular arrhythmias and an increased risk of sudden death and pathologically by fibrofatty replacement of the myocardium. Soon after the initial description of the disease, investigators and clinicians caring for patients with ARVD/C recognized a link between exercise and ARVD/C. In this article, we review the considerable body of epidemiologic data, basic research, and clinical research supporting the link between exercise and the development and outcomes of ARVD/C. Based on these data, we now advise that patients with ARVD/C avoid participation in competitive athletics and limit exercise as much as possible.