Negative pressure pulmonary hemorrhage (NPPH) is a rare complication of acute upper airway obstruction that is recognized most frequently postextubation. Ankylosing spine hyperostosis (ASH) is characterized by anterior and lateral ossification of the vertebral body, which is a rare cause of upper airway obstruction. A 73-year-old man was brought by ambulance because he felt shortness of breath with sudden diffuse bilateral ground-glass opacity and a consolidation and tracheal obstruction resulting from a large anterior osteophytic ridging at C7-Th1 level, a finding compatible with ASH. Mechanical ventilation was required, and bronchoscopy examination revealed severe tracheal stenosis and bloody bronchoalveolar lavage fluid. We diagnosed him as having NPPH associated with tracheal obstruction as a result of ASH. Resection of the osteophyte resulted in complete resolution of tracheal obstruction and symptoms. This is the first known report of NPPH resulting from ASH.