A 3-year-old boy presented with severe airway obstruction which was diagnosed as asthma. He improved but had repeated episodes of severe airway obstruction. On clinical examination, he had a tracheal cough and monophonic wheezing. Imaging revealed a large lesion in the distal part of the trachea which was confirmed by bronchoscopy. The lesion was surgically removed. Histology revealed features characteristic of an inflammatory myofibroblastic tumour. Following the resection there is no recurrence of the lesion.