A 15 1/2-year-old boy died suddenly while swimming. He had a family history of sudden death involving three consecutive generations, including a brother. The patient had a history of exercise-related syncope, for which he was being treated with nadolol. Autopsy showed enlarged heart, normal coronary arteries, right ventricular septal hypertrophy, quadricuspid pulmonary valve, accessory tricuspid valve, and a moderately elongated and thickened mitral valve. Conduction system revealed that the penetrating bundle was pushed to the left side of the summit of the ventricular septum by the right ventricular septal hypertrophy; it was lobulated and showed fatty-fibrous changes. These findings extended throughout the beginning of the bundle branches. We conclude that in this patient with familial sudden death and normal QT interval, the abnormal right ventricular septal hypertrophy altered the course and produced degenerative changes in the conduction system, which may have caused sudden death.