Résumé : Arrythmogenic right ventricular dysplasia (ARVD) is rare disease but it is a major cause of sudden death in young people and in athletes. ARVD is a cardiomyopathy characterized by structural and functional abnormalities of the right ventricle precipitating its electrical instability. This electrical instability is responsible for ventricular arrhythmias. Sudden death, by ventricular fibrillation, may be the first symptom of the disease. The diagnosis is based upon specific ventricular pathology at the histological level as well as typical electrocardiographic and diagnostic imaging features. A better understanding of the disease has increased along with the new data on genetics. Its management remains a challenge because of the wide spectrum of clinical presentation as well as its natural history. We present a case of ARVD and its evolution in order to show the difficulties of the management of this particular entity.