It is presented 40-years experience of tracheal adenocystic carcinoma diagnosis and management in 144 patients. Peculiarities of clinical course and choice of diagnostic and therapeutic measures are discussed in relation to stenosis and hypoxia degree and severity of accompanying inflammatory complications of trachea, bronchi and lungs. Technical tools and original methods of resection and reconstruction of trachea and it’s bifurcation after extended lung resection are described. Surgical management is carried out in 96 patients: all patients underwent circular resection of trachea (77) or it’s bifurcation (19). Morbidity rate was 22.9% and mortality — 3.1%. Additional radiotherapy was carried out in 50 patients. 41 patients underwent radiotherapy alone. Radiotherapy improves remote results of management. 5-and 10-year survival rate was 78.3±6.1 and 45.9±7.9% after surgery alone, 92.0±3.9 and 77.1±6.5% after combined treatment and 76.4±6.5 and 55.0±10.7% after radiotherapy alone respectively.