Idiopathic ventricular tachycardia is widely believed to carry a favorable prognosis, although there have also been reports of sudden cardiac deaths. We present a case of sudden death in a patient with apparent idiopathic right ventricular tachycardia. This patient had long-standing and exercise-related symptoms, an essentially negative non-invasive cardiac evaluation, and spontaneous and inducible ventricular tachycardia of left bundle branch block and inferior axis morphology, that was treated with propranolol. After an uneventful 5-year course, the patient died suddenly. Postmortem examination revealed a severely dilated right ventricle and significant replacement of the right ventricular wall with adipose tissue. Interstitial fibrosis was also seen, but only to a very slight degree.