Arrhythmogenic right ventricular cardiomyopathy is a hereditary myocardial condition in most cases that affects the right ventricle, but also the left ventricle with variable degree. It predisposes patients to ventricular arrhythmia, heart failure and sudden death. Its diagnosis remains challenging and is mostly based on reference task-force criteria. The latter, divided between major and minor criteria, include structural abnormalities (visualized on echocardiography or cardiac magnetic resonance), electrocardiographic anomalies, ventricular arrythmia documentation, histological proof of fibro-fatty infiltrates within myocardial tissue and family history. Following a correct diagnosis, patient-tailored care is essential. First, implantation of an implantable cardioverter-defibrillator is recommended in case of history of sudden death, sustained ventricular tachycardia or advanced right/left ventricular dysfunction. It should be considered in case of cardiac syncope or non-sustained ventricular tachycardia. Secondly, eviction of high intensity physical activity is mandatory. Finally, beta-blockers are recommended for all patients with clinically manifest arrhythmogenic right ventricular cardiomyopathy.