A 22 months old infant with agenesis of right pulmonary artery, without other associated congenital heart defects, is reported. Reconstruction of pulmonary arteries by connecting them through a 7mm Goretex tube was performed, with clear clinical improvement in view of disappearance of signs of cardiac insufficiency and of hypoxemia. Contralateral pulmonary hypertension, at systemic level, decreased to 77% in the immediate postoperative period. So, this technique becomes the first option, before considering these cases for cardiopulmonary transplantation.