We present a case of pulmonary sling associated with tracheobronchial stenosis, and with hypoplasia of the right lung and right pulmonary artery. Radiologic studies showed evidence of pulmonary sling and hypoplasia of the right lung; associated hypoplasia of the right pulmonary artery was also present, but not recognized initially. Narrowing of the distal trachea and right main bronchus was present, not due to compression by the anomalous left pulmonary artery, but to congenital stenosis of the trachea and right main bronchus associated with complete cartilaginous tracheobronchial rings.

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