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To the Editor: Persistent left superior vena cava (PLSVC) with the absence of right superior vena cava (RSVC) is a very rare congenital anomaly that can complicate the implantation of a permanent pacemaker lead. If the atrial lead is placed beyond the right atrial appendage during atrial fibrillation (AF), assessment of adequate lead fixation might be challenging. We present a case of PLSVC with the absence of RSVC and sick sinus syndrome with paroxysmal AF, in which a permanent pacemaker was successfully implanted.