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Plastic bronchitis (PB) is a rare disorder in children but a known complication of Fontan palliation. It is characterised by the formation of muco-cellular casts inside the tracheobronchial tree, which can cause recurrent life-threatening airway obstruction. Common presentation of PB includes atelectasis and dyspnea with potentially fatal outcome. The pathogenesis of this condition is not thoroughly understood. However, the abnormalities of lymphatic vessels may play a role. Various treatment strategies have been anecdotally reported but only few treatment options have been shown to be effective. Authors present a case of a five-year-old girl with Fontan physiology who developed plastic bronchitis. Symptoms occurred 15 months after third stage of correction. Treatment with mucoactive agents and inhaled bronchodilatators had no beneficial effect. A trial of inhaled alteplase was administered and resulted in a full symptomatic recovery. This kind of treatment is unregistered in Poland. The pathophysiology, clinical manifestation and therapeutic opportunities for PB in patients after cardiac surgeries have been reviewed.