Background: Tracheostomy is an essential procedure in cases of respiratory failure in patients requiring long-term ventilation or showing airway obstruction. Tracheostomy has both immediate and long-term complications. Among these, tracheo-innominate fistula is an emergency that is a rare long-term complication. When it occurs, this event is catastrophic for the patient’s life, as it causes death in a very short time due to hemorrhagic shock. Therefore, it is essential to identify risk factors to prevent these cases. Methods: We describe the autopsy findings in a case of death from tracheo-innominate fistula of a patient admitted to the Intensive Care Unit. Results: The autopsy demonstrated, in addition to the large fistula, the coexistence of a malformation of the cervical spine with a significant increase in the diameter of the neck. Therefore, we emphasize in this case the importance of evaluating risk factors in subjects with tracheostomy by highlighting the role of anatomy and the size of the neck as potential predictable risks. Conclusions: The work retraces through a review the pathogenesis of this rare complication and emphasizes the need for early diagnosis and prevention of the risk of death with specific risk scales.