Congenital complete heart block (CCHB) occurs because of transplacental antibodies such as anti-SS-A and anti-SS-B. CCHB is a potentially fatal condition, in particular in very/extremely low birth weight infants. Coexisting structural heart disease is another significant risk factor for CCHB. The survival rates after pacemaker implantation, either temporary or permanent, remain low. To the best of our knowledge, no criteria have been established for the minimum weight and earliest gestational age for treatment thus far. Herein, we present a case of an extremely low birth weight infant (ELBWI) with CCHB caused by positive anti-SS-A/Ro antibodies of the mother and significant pulmonary valve stenosis. The infant underwent temporary pacing implantation on the day of birth with a weight of 850 g. Percutaneous balloon pulmonary valvuloplasty was performed at the age of 106 days when body weight 2.6 kg. This consecutive approach provided a favorable outcome. We propose that epicardial temporary pacing could avoid postnatal heart failure in ELBWI with CCHB even when structural heart disease was present.