Arrhythmogenic right ventricular cardiomyopathy (ARVC) is a genetic heart condition that causes progressive fibrofatty replacement of the myocardium, leading to ventricular tachycardia and sudden death, primarily affecting the right ventricle. Diagnosis involves a combination of noninvasive and invasive tests, while management is individualized, focusing on prevention of sudden death through medication and an implantable cardioverter-defibrillator, and avoiding regular, vigorous athletic activity.