BackgroundDieulafoy’s disease is a vascular anomaly characterized by the presence of a tortuous dysplastic artery in the submucosa. Although frequently occurring in the gastrointestinal tract, multiple cases of Dieulafoy’s disease in the bronchus have been reported in the literature.Methods and resultsWe report a case of a 15-year-old boy suffering recurrent massive hemoptysis. Bilobectomy stopped bleeding after unsuccessful treatment with embolization of bronchial artery.ConclusionIt is concluded a congenital origin of this disease. Angiography and endobronchial ultrasonography can be used to diagnose Dieulafoy’s disease of bronchus whereas bronchoscopy biopsy should be avoided. Surgery is needed when embolization fails.Electronic supplementary materialThe online version of this article (doi:10.1186/s13019-014-0191-8) contains supplementary material, which is available to authorized users.