Superior vena cava (SVC) syndrome after pacemaker implantation is a very rare complication. We present a 56-year-old man with severe swelling of the face, neck, and upper arms about 6 years after pacemaker implantation. Digital subtraction angiography showed occlusion of the left innominate vein and severe stenosis of the SVC. The patient eventually required surgical treatment, since anticoagulant therapy and balloon venoplasty were not effective. We conclude that surgical repair is the most effective method for treating similar patients.