Cystic fibrosis (CF) is a fatal genetic disorder that affects many organ systems in the body. Historically, few patients with CF lived beyond early childhood, but with continuous improvement in treatment modalities, quality of life and the life span of persons with CF has greatly improved. As the surviving population of people with CF increases, a greater chance of encountering them in anesthesia practice exists. Comorbidities associated with the disease, such as diabetes mellitus and osteopenia, may also contribute to an increased frequency of surgical and anesthetic encounters. An understanding of the pathophysiology of the disease, as well as anesthetic implications and management, is crucial to the safe administration of anesthesia in this population. Cystic fibrosis is traditionally thought of as a childhood disease affecting the lungs and pancreas, which does not accurately describe the disease in its entirety. Many organ systems are affected, from the heart and lungs to the reproductive system, and may warrant alterations in an anesthetic plan. This review highlights the pathologic conditions associated with multiple systems, therapy regimens, and potential complications and suggests anesthetic implications.