We present a case of successful implantation of a permanent pacemaker through an unusual course of a persistent left superior vena cava (PLSVC). A young male patient presented with symptomatic bradycardia and a heart rate of 35 beats per minute. The pacing lead was introduced through the standard left subclavian vein approach and was found to pass through an unusual course that was suspected to be a PLSVC. The lead was further advanced to the right atrium and positioned successfully in the right atrial appendage with excellent short-term and long-term pacing parameters. PLSVC should be suspected whenever the catheter takes an unusual position during central venous catheterization. Most right heart catheterization procedures, including permanent pacemaker implantation, can be safely completed in spite of this anomaly.