The esophageal lung is a rare congenital malformation of the foregut, characterized by the existence of the lung tissue, a segment of the lung, and the main bronchus between the esophagus and the part of respiratory system. Because of the rare occurrence of this defect, examples of the treatment of this disease can be found only in foreign literature, in Russian-language sources, there are no described cases of treatment of the esophageal lung. In this regard, the literature data was analyzed and there was presented, clinical picture, classification, diagnosis, and treatment of this defect, as well as a case from practical activities was carried out: diagnosis and treatment of a premature one month baby with an esophageal lung.