Persistent left superior vena cava (PLSVC) with absence of right SVC (isolated PLSVC) is a rare congenital anomaly that occurs as a result of a degenerative condition in the left anterior cardinal vein. It is generally an incidental finding while performing invasive procedures such as antiarrhythmic device implantation. We report on a rare case of permanent pacemaker implantation in a patient with this anomaly from right subclavian route, albeit most of the earlier reported cases are from left subclavian approach. A wide spectrum of clinicians should be aware of this anomaly, its variations, and possible complications.