We report on a 48-year-old man who presented with recurrent sustained monomorphic ventricular tachycardia, which resulted in syncope on one occasion. Subsequent investigation confirmed the diagnosis of arrhythmogenic right ventricular dysplasia. Familial screening for the disease was conducted using 12-lead electrocardiography, signal-averaged electrocardiography, and magnetic resonance imaging. Two of the four relatives who were screened showed evidence of the disease, thus confirming the familial form of arrhythmogenic right ventricular dysplasia in the patient. He underwent a subpectoral implantation of an implantable cardioverter defibrillator in view of the malignant nature of the ventricular tachycardia and his intolerance to drug treatment.