Arrhythmogenic dysplasia of the right ventricle is a degenerative disease. Accurate incidence of the disease is unknown. The highest incidence of ARVD/C is in families with affected patients. ARVD/C is an autosomal dominant or recessive type of heredity. Sporadic forms of ARVD/C are not rare. Clinical picture is poor. The first clinical sign is sudden heart death. Even asymptomatic patients are at risk of fatal arrhythmias. Prognosis of treated patients is rather good. ICD implantation has reduced mortality of affected patients.