Superior vena cava syndrome as a complication of permanent transvenous endocardial pacing is unusual. We herein report on a 71-year-old woman who presented with progressive swelling of the face, neck, thorax and upper limbs two months after implantation of a permanent transvenous endocardial pacing device for sinus node dysfunction. Superior vena cava syndrome was diagnosed by venography. Acute heart failure and respiratory failure developed before her scheduled operation. She expired despite vigorous cardiopulmonary resuscitation.