A 56-year-old man with Brugada syndrome presented with ventricular fibrillation induced by administration of pilsicainide. He had syncope at age 46 years, and his uncle suddenly died of unknown cause. He had taken pilsicainide (150 mg/day) for paroxysmal atrial fibrillation, and suffered from syncope due to ventricular fibrillation. Coved type ST elevation was observed in the V1 lead, and saddle back type ST elevation was observed in the V2 lead. The ST elevation gradually recovered after stopping pilsicainide therapy. No structural heart disease was found. After intravenous injection of pilsicainide, the ST segment elevated in the V1 and V2 leads. Ventricular fibrillation was induced by triple extrastimulation at the right ventricular apex. The diagnosis was Brugada syndrome, and a cardioverter-defibrillator was implanted. Brugada syndrome should be considered before administering pilsicainide.

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