We report a 43-year-old woman with familial amyotrophic lateral sclerosis (FALS) who died of massive bleeding from a tracheoarterial fistula. Four years after the onset of the disease, she received invasive ventilation by tracheostomy because of respiratory failure. Four years and 7 months later, she showed an abrupt hemorrhage from the tracheostomy and died. The postmortem examination revealed a fistulous tract between the tracheal mucosal ulcer and the brachiocephalic trunk. The ulcer was in close proximity to the tracheostomy tube. In order to avoid such unexpected complications, we should observe the contact site between the tracheal mucosa and the tracheal tube in chronic tracheostomy patients.