Ankylosing spondylitis is a chronic inflammatory disease that primarily affects the joints of the axial skeleton. Pleuropulmonary involvement is an uncommon, late event in the course. A 53-year-old man who had a diagnosis of ankylosing spondylitis since he was 40 years old developed a bilaterally repeated and refractory spontaneous pneumothorax. He was treated successfully with surgery to the left pneumothorax that had been refractory to conservative chest tube drainage and chemical pleurodesis. During the second episode of right-side pneumothorax, he developed severe respiratory insufficiency because of his coexisting restrictive lung disease. He was successfully treated with chemical pleurodesis to the right pneumothorax. In our experience, prophylactic treatment such as surgery and pleurodesis should be considered for patients with ankylosing spondylitis during the first episode of pneumothorax.