Vascular compression of the airway is a significant cause of respiratory compromise in children. While the indications for surgical repair are sometimes life threatening, they can also be subtle. This retrospective study examines 45 surgical cases of tracheobronchial compromise secondary to vascular compression at a large children's hospital between July 1983 and February 1996. A total of 34 were diagnosed with innominate artery compression, ten with a double aortic arch and one with an anomalous right subclavian artery. The 45 patients, 25 male and 20 female, ranged in age from 12 days to 11 years at surgery (average 13 months). A total of 21 (47%) presented with proven or suspected episodes of cyanosis or apnea. All 45 patients had evidence of vascular compression during microlaryngoscopy and bronchoscopy. The diagnosis was confirmed by magnetic resonance imaging (MRI) in 23/45 (51%), barium swallow in 22/45 (49%) and aortogram in 3/45 (7%). There was one death. One patient had a tracheotomy before surgery and continues to require it after surgery. Complete resolution of symptoms was achieved in 39/45 (87%) with five requiring more than one operation before their symptoms resolved completely. A total of four patients experienced a recurrence of symptoms within a variable length of time after surgery. Surgical indications and treatment alternatives will be discussed.