Context Hemorrhage is one of the potentially fatal complications of tracheostomy. A rare but lethal cause of tracheostomy related bleeding is hemorrhage from the innominate artery. This occurs following tracheo-innominate artery fistula (TIF) formation, which is associated with a mortality rate of more than 85%. Here, we report the case of an individual with tetraplegia and a tracheostomy who died as a result of innominate artery hemorrhage. This case highlights the possible causes and interventions associated with this complication, and provides insight into tracheostomy related bleeding in patients with spinal cord injury (SCI). Findings A 15-year-old boy with a diagnosis of incomplete SCI at the C5 level was admitted for rehabilitation 4 months after injury. He required a tracheostomy for ventilation, and underwent subglottic stenosis dilatation thrice. Multiple decannulation attempts were performed without success. He received intensive care on several occasions for respiratory failure. During the course of his rehabilitation, a minimal tracheostomy bleed was observed, which became profuse within a few hours and led to hypoxia with loss of consciousness. An urgent sternotomy identified bleeding from a TIF. He suffered severe brain damage following massive tracheal hemorrhage and died. Conclusion/clinical relevance Given the morbidity of TIF-related hemorrhage, it is important to increase awareness of this rare condition among health-care providers, especially those in non-acute settings. Patients with SCI and a tracheostomy pose unique challenges related to respiratory compromise, which may accentuate TIF formation.