Diffuse idiopathic skeletal hyperostosis (DISH) is a rare and potentially life-threatening syndrome. We present the case of a patient complaining about severe dyspnoea and diagnosed with vocal cord paresis. An emergency tracheotomy was performed to restore his breathing. Diagnostic imaging revealed large massoccupying cervical osteophytes compressing the larynx. The osteophytes were removed via an anterior cervical approach, and vital signs were normalized. However, postoperatively, a fistula was discovered between the upper part of the oesophagus and the trachea. As a result, a gastrostomy tube had to be placed indefinitely. Literature review confirms the rare frequency of emergency tracheostomy due to DISH syndrome. The aim of the present study is to expand on our knowledge of a rare pathological entity that can frequently be misdiagnosed.