Introduction: Our previous study showed that arrhythmogenic right ventricular cardiomyopathy (ARVC) is an important cause of sudden unexplained death in Korea. However, clinical characteristics of Korean patients with ARVC are not known well. Methods: Eighty-nine patients with nonischemic wall motion abnormality and/or ventricular arrhythmia were studied by routine ECG, 2-D echo, exercise ECG, Holter ECG, signal-averaged ECG, coronary angiography, right ventriculography and endomyocardial biopsy. Clinical data of patients who met the Task Force Criteria for ARVC were analyzed. Eleven patients with typical features of dilated cardiomyopathy on endomyocardial biopsy were excluded from further analysis. Results: Finally 24 (eleven males) patients were diagnosed with ARVC. Mean age at clinical presentation was 45짹12 years. Three patients (12%) had family history of premature sudden death including 1 autopsy proven case of ARVC. The presenting symptoms were palpitation (33%) and syncope (37%), atypical chest pain (12%) and no symptom (12%). Sustained ventricular tachycardia was the presenting arrhythmia in 7 (29%) and no significant ventricular arrhythmia was detected in 8 (33%). Echocardiography and/or right ventriculography showed wall motion abnormality of right ventricle in 22 (91%). Endomyocardial biopsy showed typical fatty or fibrofatty infiltration in 21 (87%). Late potentials were positive in 12 (50%). Most patients were treated with beta blocker and/or amiodarone. Implantable cardioverter defibrillators were implanted in 2 patients. During a mean follow-up of 30짹27 months no syncope, sudden death or shocks were developed. Conclusion: ARVC may be an important cause of ventricular arrhythmias and/or nonishemic wall motion abnormality of right ventricle in Korea. However, some patients were not typical in clinical presentation. Extensive studies may needed for the proper diagnosis of ARVC patients with atypical or no symptoms.