Cardiac surgery for very-low-birth-weight infants is rarely reported, especially for a triplet. We herein report the successful staged repair of a premature triplet accompanied with transposition of the great arteries. During pregnancy, the fetuses were diagnosed as dichorionic diamniotic triplets, and the mother entered a hospital for maternal protection and health care from 25 weeks' gestation. The triplets were delivered by Caesarean section at 33 weeks and 5 days of gestation because of intrauterine growth retardation. One of the infants, weighing 1,336 g, was diagnosed with transposition of the great arteries (type II). Since he was deemed unable to endure an intracardiac repair, he received balloon atrial septostomy on the 27th day of life and then bilateral pulmonary artery banding on the 29th day of life. However, further balloon atrial septostomy on day 69 and left pulmonary arterial de-banding on day 73 post-birth were needed because of the progression of hypoxia. He received prolonged intubation and inotropic support after temporary cardiopulmonary stability, and we ultimately decided to perform arterial switch operation on day 110, when he weighed 1,838 g. The patient showed a good recovery. In the field of pediatric cardiac surgery, we occasionally select staged strategies for patients who cannot undergo radical operations all at once because of their general condition or low body weight. However, there are no established guidelines concerning the timing of palliative or radical operations in low-birth-weight infants. At present, we select medical strategies ourselves, on a case-by-case basis. In the present case, although our medical strategy had to be adapted, we still obtained a good recovery for this triplet with extremely low birth weight. We herein report this case with some references from the literature.