Eight patients are presented who demonstrate many of the pulmonary complications seen in the prune-belly syndrome. The patients are divided into two major groups: Group I includes pulmonary hypoplasia; Group II includes lobar atelectasis and pneumonia. The etiology, pathogenesis, and radiographic features of these complications are discussed. Pulmonary complications become more important as renal dialysis and transplantation spare more of these patients from an early uremic death. Prompt recognition of the type and the extent of pulmonary disease in patients with the prune-belly syndrome may lead to increased survival.