Persistent left superior vena cava (LPSVC) is a rare congenital anomaly of the thoracic venous system that can trigger paroxysmal atrial fibrillation. The role of this venous anomaly must be carefully considered in patients undergoing conventional atrial fibrillation transcatheter ablation by pulmonary vein isolation to avoid unnecessary lesions, left atrium access and arrhythmia relapses. In fact, the present clinical perspective suggests sole LPSVC isolation is a well tolerated and effective approach in patients with paroxysmal atrial fibrillation and arrhythmic trigger originating from a LPSVC.