This review is an attempt to summarize relevant gastrointestinal surgical issues in the patient with cystic fibrosis. Many of the surgical treatments are standard and have remained unchanged for several years and are only briefly discussed. A few areas with new developments are meconium ileus and the implications of prenatal diagnosis of meconium peritonitis. In addition, new findings with hepatobiliary disease and gastroesophageal reflux disease associated with cystic fibrosis patients may change the manner in which these entities are approached because these patients are now living longer. Finally, we review the recent findings associated with fibrosing colonopathy.