In patients with situs inversus totalis, the superior vena cava is normally positioned on the left side and drains into a left-sided right atrium (RA). If right-side superior vena cava (RSVC) is also present, it should be thought of as a combined congenital anomaly. Here, we report a case of successful pacemaker lead insertion through the RSVC in a patient with situs inversus totalis. The left-side superior vena cava (LSVC) had been already used as a route for the first pacemaker lead insertion 15 years earlier. During the pacemaker lead revision, we found that the LSVC was obliterated, and used the RSVC as a route for a new pacemaker lead insertion.