This review discusses current aspects of the diagnosis and treatment of adenoid cystic carcinoma of the trachea and larynx. It describes different variants of surgical treatment, radiotherapy, combination therapy, endotracheal interventions using endoscopic techniques and physical methods, grafting, tracheal transplantation, complications, and oncological and functional treatment outcomes. We also analyze clinical aspects of adenoid cystic carcinoma of the larynx, most common tumor location, difficulties associated with morphological verification, and controversial issues related to surgery volume, considering that this disease is rare and clinical experience is often insufficient.