AbstractArrhythmogenic right ventricular cardiomyopathy (ARVC) is a progressive cardiomyopathy characterized by fibrofatty infiltration of the myocardium, ventricular arrhythmias, sudden death, and heart failure. ARVC may be an important cause of syncope, sudden death, ventricular arrhythmias, and/or wall motion abnormalities, especially in the young. As the first symptom is sudden death or cardiac arrest in many cases, an early diagnosis and risk stratification are important. Recent advances in diagnostic modalities will be helpful in the early diagnosis and proper management of patients at risk. Restriction of strenuous exercise and implantation of implantable cardioverter‐defibrillators are important in addition to medical treatment and catheter ablation of ventricular tachycardia. Recently introduced genetic screening may help to identify asymptomatic carriers with a risk of a disease progression and sudden death.