BACKGROUND Congenital tracheomalacia (CTM) is a rare disease causing tracheal wall collapse when breathing. Herein, we show our experience in the management of this type of airway anomaly, settling the indications for surgical or endoscopic treatment. METHODS We have performed a retrospective study, from 1991 to 2003, of patients with a bronchoscopic diagnosis of CTM or bronchomalacia (BM). We have analyzed the following facts: sex, age, indication of the initial bronchoscopy, ethiology, clinical group, anatomic type, associated malformations, treatment modality, complications, results, and time of follow-up. RESULTS 46 patients have been included in this study: 25 boys (54%) and 21 girls (45%). Mean age at diagnosis has been 11 months. The indications for diagnostic bronchoscopy have been: respiratory distress (24%), lung athelectasia (24%), stridor (21%), congenital tracheoesophageal fistula (11%), extubation failure (11%), apneic spells (6%), and recurrent pneumonia (2%). Secondary CTM has been much more frequent (82%) than the primary type (17%). Patients have been classified into 3 groups according to the severity of symptoms: group I--mild symptoms (7 patients); group II---moderate (22); and group III, severe (17). Tracheomalacia was diagnosed in 26 cases (56%), bronchomalacia in 12 (26%) and tracheobronchomalacia in 8 patients (17%). Almost all the patients (95%) have showed other associated malformations. Medical treatment has been instituted in 29 patients (63%), 15 cases (32%) have been managed surgically or endoscopically, and in 2 cases no treatment was tried because of their critical clinical status. In addition, in 17 patients (37%) an antireflux surgical procedure was performed. Satisfactory results have been achieved in 72% of treated patients, fair results were obtained in 4 (9%), and a poor outcome occurred in 2 (4,5%). Another 8 patients have died during follow-up due to unrelated causes. 36 patients (78%) are alive with a mean follow-up period of 5,3 years. CONCLUSIONS Most patients with CTM can he treated conservatively though spontaneous resolution may he expected after the first year of life. Surgical or endoscopical procedures are indicated in those patients with severe respiratory symptoms.