Pulmonary atresia with intact ventricular septum is difficult to manage, and the surgical procedures are associated with high mortality and morbidity. The lesion is morphologically heterogeneous, with varying degrees of right ventricular and tricuspid valve hypoplasia. Aberrations of the coronary circulation are common. A single approach is impractical because the wide spectrum of right heart morphology makes a uniform surgical approach impossible. Anatomical criteria, such as the degree of tricuspid valve or right ventricular hypoplasia or whether a right ventricular infundibulum is present, have been used to assign the infants to single-ventricular or biventricular repair tracks. If the patient is considered a candidate for biventricular repair, right ventricular decompression, either alone or combined with a systemic-pulmonary artery shunt, is performed, whereas only a systemic-pulmonary artery shunt is performed in a candidate for single-ventricular repair. Several authors reported that the Z-value of the tricuspid valve diameter could be a guide to the initial procedure; however, poor interobserver reproducibility and wide normal ranges are serious limitations. Definitive operations, including biventricular repair, one-and-a-half ventricular repair, or Fontan-type operation, are performed to eliminate cyanosis. The most important problem when planning a strategy for a definitive repair is the fact that right ventricular growth and its contribution to pulmonary flow is difficult to predict before the operation. The management protocol should be determined by the precisely estimated morphology of the right ventricle and tricuspid valve, and the adequacy of the protocol should be analyzed based on surgical outcomes.