Background: The clinical characteristics of the Korean patients with arrhythmogenic right ventricular cardiomyopathy (ARVC) are not known well. Methods: The clinical data of the patients who met the Task Force Criteria for ARVC were analyzed. Results: Thirty-seven patients (41짹15 years old, 19 males) were diagnosed with ARVC. Three patients had a family history of premature sudden death (SD). Their presenting symptoms were palpitations (n=11), syncope/presyncope (n= 11), atypical chest pain (n= 3), cardiac arrest (n=1), SD in the family (n=1) and no symptoms (n= 10). Ventricular tachycardia was observed in 13 and no significant ventricular arrhythmias were detected in 14. Wall motion abnormalities of the right ventricle were detected in 35. Endomyocardial biopsy revealed fibrofatty infiltration in 26 of 32 patients who underwent endomyocardial biopsies. Most patients were treated with 棺-blockers and/or amiodarone. Two patients had symptoms of heart failure and were treated accordingly. Implantable cardioverter-defibrillators (ICDs) were implanted in 3 patients. During a mean follow-up of 28짹26months no syncope or SD developed except for in 1 patient with an ICD who suffered from recurrent shocks due to ventricular fibrillation. Conclusions: ARVC may be an important cause of nonischemic wall motion abnormalities of the right ventricle, and/or ventricular arrhythmias in Korea. Some patients did not have a typical clinical presentation.