This is a collective review of 59 published cases of right pulmonary artery to left atrium communication in the English-language literature. In this article, we review the literature on the clinical presentation, anatomic variations, diagnostic features, and management considerations of previously reported cases. Two-dimensional echocardiography and cardiac catheterization including selective angiography provided the necessary diagnostic information and defined the anatomy before surgery in all patients. Various management options including medical, surgical, and transcatheter closure have been reported. The lesions have been classified into four types (I, II, III, and IV) that have a bearing on the appropriate surgical approach and techniques of repair that are discussed in the text. Use of extracorporeal circulation may be considered in selected instances. The operative mortality rate during the earlier era (1950 to 1979) was high, at 22%. More recently, the mortality rate has approached zero. An individualized surgical approach depending on the type of surgical connections is recommended.