The patient was a child born by cesarean section at 37 weeks gestational age, weighing 2,340 g, measuring 44 cm, with a cephalic perimeter of 32 cm; the 1 and 5 minute Apgar scores were 8 and 9. The mother was primiparous, aged 24 years at the time of birth, whose parents were second degree cousins; there was no family history of genetics diseases. Severe micrognathia, a cleft mandible, and altered clavicle and lower/upper limbs were found on the physical examination. Nasofibrolaryngoscopy revealed a retropositioned tongue that occluded the glottis; the epiglottis was absent and there was severe laryngomalacia that resulted in respiratory failure. A tracheostomy was carried out because of this condition, and the patient remained in hospital until the seventh month of life; during this period the patient had several respiratory events. While still in hospital, mandible distraction surgery followed by cranial bone grafting to anteriorize the mandible and unobstruct the airways for removal of the tracheostomy tube was done. These procedures proved insufficient to maintain the airways pervious, and the patient remained with the tracheostomy tube. Placing a fenestrated tube with a speech valve was also unsuccessful, as the cleaning procedures caused excessive and repeated bleeding, and a suprastomal granuloma due to long term use of the tube resulted in obstruction of the stoma, which made inspiration even more difficult. At age 2 years and 4 months, an external compression of the main left bronchus was observed in a routine bronchoscopy. Computed tomography of the thorax and mediastinum revealed a mediastinal mass that exerted pressure on the main left bronchus; a biopsy resulted in a diagnosis of tuberculosis, which was treated successfully. At present, every two months, the metal tracheostomy tube is changed and bronchoscopy is carried out to assess the airways and, if necessary, to cauterize the suprastomal granuloma. Throughout this period, retrognathism and retropositioning of the tongue have remained unchanged; the tongue rests over the vocal folds (the epiglottis is absent), which maintains the airways obstructed (Fig. 1) and precludes removal of the tube.