Clinical, radiologic and histologic features of obliterative bronchiolitis (OB) in children were reviewed to define features helpful in early recognition. All autopsies (n = 2,897), lung biopsies (n = 244), and medical records of children followed at St. Christopher's Hospital for Children (SCHC) between 1960 and 1985 were screened. Nineteen cases of OB were confirmed using radiologic and histologic criteria. Twelve patients were diagnosed during life, and ten survived. Seven were diagnosed post mortem. OB should be considered when persistent respiratory signs and symptoms follow acute pneumonia, aspiration is known or suspected, areas of hyperlucency are seen on chest radiograph, or respiratory failure with overaeration is unresponsive to therapy. Diagnostic studies include ventilation-perfusion scan, bronchography and lung biopsy. Sequelae include dyspnea on exertion, obstructive lung disease, bronchiectasis, persistent atelectasis, and hyperlucent lung syndrome. Recognition and supportive treatment during the acute and chronic phases may improve the functional status of these patients.