A case is presented of an 18-year-old male who had been resuscitated following an episode of sudden death due to ventricular fibrillation. The patient was noted to have an abnormal deflection in the terminal QRS on surface ECG and an abnormal signal-averaged ECG demonstrating a late potential coincident with the terminal QRS abnormality on the ECG. The patient had easily inducible polymorphic ventricular tachycardia during electrophysiologic study, which was suppressed by quinidine but not by procainamide or beta blockers. The surface ECG and signal-averaged ECG also were normalized by quinidine but not by procainamide or beta blockers. The patient had no further arrhythmias on quinidine for 6 years until he inexplicably discontinued his medication and died suddenly shortly thereafter. The present case may represent a unique familial sudden death syndrome or possibly a variant of the sudden death syndrome associated with right bundle branch block and ST elevation in V1 through V3. Currently available data suggest that, in such patients, an implantable cardioverter defibrillator may provide better protection from sudden death than does antiarrhythmic drug therapy.