Tracheostomy is required in approximately 22% of children with Goldenhar syndrome because of upper airway obstruction. We report a case of problematic tracheostomy placement in a 24-week-old ex-premature infant with Goldenhar syndrome. This infant had potentially life-threatening ventilatory insufficiency following tracheostomy placement related to tracheostomy tube size and position. These complications may have been avoided by a change in practice in methods of assessment of tube position.