A 43-year-old man with a 30-year history of WPW-syndrome and a hypertrophic cardiomyopathy developed acute heart failure after onset of atrial fibrillation with fast antegrade conduction, which could be converted to sinus rhythm with antiarrhythmic medication. Catheterization of the coronary sinus during EP testing demonstrated a persistent left superior vena cava. The accessory pathway could be localized at the orifice of an atypical epicardial vein. It was successfully abolished after subvalvular placement of the electrode catheter in the left ventricle. This constellation indicates a combined defect during the regression of the sinus venosus to the sinus coronarius with persistence of conducting muscle fibers. Successful RF ablation procedure provides an obvious risk reduction as a result of a lower frequency of atrial fibrillation and the eliminated risk of ventricular fibrillation due to rapid conduction via an accessory pathway. Beyond that, harmless therapeutic treatment of hypertrophic cardiomyopathy with a calcium-channel-blocker (verapamil type) can follow RF ablation.