Anomalous inferior vena cava without intracardiac anomaly is an unusual condition. Herein, we report a 48-year-old female with left-sided inferior vena cava and azygous continuation. accompanied by sick sinus syndrome. This anomaly resulted in difficulty in implanting a traditional hook-on atrial lead. Atrial lead dislodgment occurred repeatedly soon after implanting the pacemaker because of an anomalous zygous vein draining into the superior vena cava, making a giant connection with the right atrium, thus eliminating the space of the atrial appendage for lead lodgment. Finally, we attempted to utilize a screw-in atrial lead in this patient and she is currently doing well. We therefore suggest that a screw-in atrial lead should be taken into account for such patients in order to obtain a stable fixation.