Early diagnosis, treatments of acute exacerbations, and chronic therapies have all improved the lifespan of cystic fibrosis patients; however, the natural history remains one of worsening bronchiectasis and obstructive airways impairment. The progression of disease leads to eventual respiratory failure, but some will have other acute respiratory complications that require intervention. In this report, we review the most common life-threatening respiratory complications of cystic fibrosis, including pneumothorax, massive hemoptysis, and respiratory failure.