It has been demonstrated that idiopathic ventricular fibrillation (VF) can be triggered by ventricular premature beats (VPBs) arising from the Purkinje fibers. Eliminating these VPBs by radiofrequency catheter ablation prevented VF recurrences. Whether the same pathophysiology and the same treatment option exist in patients with structural heart disease is unknown. Recurrent VF was observed in a 17-year-old patient after aortic valve repair of a perforated noncoronary cusp with resulting severe aortic regurgitation. VF recurred despite therapy with various antiarrhythmic drugs. A maximum of 14 external defibrillations was necessary during a 24-hour period to stabilize the patient. Due to increasing hemodynamic instability as a result of this electrical storm, the patient was referred for invasive diagnostics. During electrophysiologic study, frequent short runs of VF initiated by VPB with a narrow QRS complex were observed. After extensive mapping of the right and left ventricles, two distinct sources of VPBs originating from anteroseptal and inferoseptal areas of the left ventricle could be successfully ablated. VPBs were preceded by distinct Purkinje potentials with intervals from the Purkinje potential to QRS onset of VPB of 68 ms and 30 ms at effective sites, respectively. During short-term follow-up of 2 months, there was no VF recurrence. VPB originating from the Purkinje system may be one possibility for VF initiation in patients with structural heart disease. Eliminating these sources of VPBs by catheter ablation can prevent recurrent VF in such patients.