Arrhythmogenic right ventricular cardiomyopathy (ARVC) is an inheritable heart muscle disease characterized by fibrofatty replacement of the right ventricle (RV) and by ventricular arrhythmias potentially leading to sudden cardiac death, mostly in young people and athletes. Later in the disease history, the RV becomes more diffusely involved and left ventricular involvement may result in biventricular heart failure. However, clinical diagnosis of ARVC is often difficult to make in the early stage of the disease because of the broad spectrum of phenotypic manifestations and the nonspecific nature of the disease features. In 1994, an international task force proposed criteria for the clinical diagnosis of ARVC, which have been recently revised to improve their sensitivity. Causative mutations have been identified in approximately half of patients with ARVC. Advances in molecular genetics of ARVC have provided important insight into our understanding of the pathogenesis and pathophysiology of ARVC, which has contributed to the improvement of clinical management. Therapeutic strategies for the prevention of sudden death and disease progression include antiarrhythmic drugs, catheter ablation, and use of an implantable cardioverter defibrillator (ICD). ICD is the most effective tool against arrhythmic sudden death. The implantation of an ICD should be carefully evaluated because of the possibility of device/lead-related complications and inappropriate interventions. This review article focuses on the most current knowledge regarding clinical presentation, diagnosis, molecular genetics, and management strategies of ARVC.