Background and aims: The incidence of congenital stenosis of the main bronchus (MBS) appears in 0.1–5% cases. In patients with congenital heart disease (CHD), the postoperative period is often accompanied by respiratory problems that may be intensified by concomitant bronchial stenosis. Aims: The study aims to determine managing tactics for the children with congenital MBS. Methods: The study included 10 children aged from 10 days to 1 year who underwent surgical correction of complex CHD in 2012–2013. Before the operation all patients breathed spontaneously, and MBS was not pre-operatively diagnosed only in 1 patient. Results: Four patients underwent balloon dilatation of the stenotic bronchus (in one - simultaneously with the correction of CHD) and the others were being managed conservatively. Postoperative respiratory disorders were observed in all patients: 6 cases of atelectasis on the affected side, 4 cases of subsequent air trapping emphysema syndrome and 5 cases of pneumonia. The average duration of mechanical ventilation was 56 days, and it was significantly increased in 50% of the ICU patients due to the MBS effects. There were 3 cases of tracheal reintubation. The ICU period was 2 - 200 days. One patient with critical respiratory insufficiency died. There were four cases of the full restoration of bronchial patency. Conclusions: Children with CHD and MBS always have respiratory problems in postoperative period that intensify the severity of their condition and significantly increase the ICU-time. It is needed to determine tactics for these patients at all stages of treatment.