We described a case of a 58-year-old man with organic changes consistent with right ventricular cardiomyopathy. He also had a loss-of-function mutation in the cardiac sodium channel gene SCN5A, described in Brugada syndrome. He first presented with non-sustained ventricular tachycardia and was implanted with an implantable cardioverter defibrillator. He remained asymptomatic for 8 years until he developed recurrent episodes of ventricular tachyarrhythmias, which required multiple shocks. The patient was treated with a combination of quinidine and verapamil and since then remained free of arrhythmias.