Spontaneous pneumothorax is a common complication in patients with cystic fibrosis (CF). It is thought to occur more frequently in patients with more advanced disease. Recommendations on the management of pneumothorax in CF are based on retrospective analyses and reports from CF centers. The following is a review of what has been published regarding the incidence and management of pneumothorax in this population, with some comment on the pathogenesis of the complication.