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Tracheal pathology in children are primarily congenital. They can be considered primary or de novo, when this is seen as an inherent defect within the cartilages of the tracheal segment. While segmental cartilage defects are very rare, there are often occasions when one or more cartilages can be considered missing from the length of trachea, contributing to airway abnormality. Secondary tracheal pathologies can often be seen in relation to disorders affecting nearby vascular elements or thoracic cage in general. In general, the pathological entity of tracheal disorders can be classified into either tracheomalacia or tracheal stenosis.

Dieulafoy's disease is a rare vascular lesion characterized by presence of large aberrant arteries within the submucosa of gastrointestinal tract or respiratory tract with a potential to cause life-threatening hemorrhage. Treatment includes bronchoscopy ablation, angiographic embolization or surgery. We report management of 7-year old girl with Dieulafoy's disease in the airway who presented with recurrent hemoptysis. Bronchial angiography revealed multiple feeding vessels to the lesion. Considering the potential risk of recurrence with embolization, sleeve resection of bronchus offered complete resolution. This case demonstrates the usefulness of bronchial angiography as part of multi-faceted approach before surgery in the management of Dieulafoy's disease.