Tracheomalacia can be a life threatening upper air way obstructive disease in an infant and vascular rings can be also a major rare cause of tracheoesophageal obstruction. These two rare entities can be combined in one patient because the vascular ring can cause secondary tracheomalacia during development of fetus. The diagnosis of this combination and adequate surgical correction is occasionally difficult. This is a report of an infant who had not diagnosed tracheomalacia associated with vascular ring until 5 months of age because of the prolonged tracheal intubation. The rigid bronchoscopic examination performed under impression of tracheomalacia revealed a concentric tracheal collapse, an unusual bronchoscopic findings of tracheomalacia, which raised a suspicion of the tracheal compression by vascular rings. The 3-D reconstructive DT aortography clearly demonstrated the double aortic arch. The patient was treated surgically by simple division of the left aortic arch and aortopexy with good result. The vascular ring such as double aortic arch should be considered during the diagnosis of tracheomalacia in infants. If the tracheomalacia is associated with vascular ring, simultaneous surgical correction should be performed.