The cystic fibrosis transmembrane regulator protein (CFTR) is an ion channel in the apical surface of epithelial membranes that regulates other ion channels. Dysfunction of CFTR leads to the clinical entity of CF when mutations in CFTR are inherited in an autosomal recessive fashion. Although airway obstruction, inflammation, and infection are usually the most serious consequences of CFTR dysfunction because they lead to respiratory failure, CFTR dysfunction affects the intestinal tract and the pancreatic and hepatobiliary ducts in a similar fashion, leading to significant morbidity. This review outlines pathophysiology and common gastrointestinal ailments in the CF population along with current medical and surgical management.