Once a death sentence, many patients with cystic fibrosis (CF) are now surviving into their forties and almost half of the current CF population in the USA are over the age of 18 years. Nutritional interventions that are aimed towards maintaining normal weight and prevention of nutritional deficiencies are critical in the management of CF and directly impact pulmonary function and survival. CF is a multisystem disease and several complications can interfere with the ability to ingest food or digest and absorb nutrients. This review investigates various gastrointestinal and endocrine complications that impact nutritional status in CF. Specific nutritional therapies are highlighted ranging from nutritional counseling to enteral and parenteral nutrition with emphasis on pancreatic enzyme replacement therapy and the challenges associated with their use.