Neurogenic tumor of the lung is very uncommon. To the best of our knowledge, endobronchial schwannoma complicated by massive hemoptysis in a patient with neurofibromatosis 2 has not been reported previously. We report a case of endobronchial schwannoma complicated by massive hemoptysis in an 18-year-old man with neurofibromatosis 2. The diagnosis of neurofibromatosis 2 was established by demonstration of bilateral vestibular schwannomas on magnetic resonance imaging of the brain and pathologic examination of the resected brain tumors. Massive hemoptysis developed after surgical removal of the brain tumors in this patient. Bronchoscopy was done immediately after the first episode of hemoptysis and a tumor protruding from the orifice of the right lower lobe bronchus was found. Despite 2 additional bronchoscopies to stop bleeding and ameliorate airway obstruction in the consecutive 2 days, hemoptysis recurred rapidly and caused profound oxygen desaturation. The patient was subjected to right lower lobectomy and endobronchial schwannoma was evidenced pathologically.