Abdominal pain is a common symptom in individuals with cystic fibrosis (CF). As prognosis has improved, CF has changed from a pediatric disease to the current situation wherein most people with CF are adults. With improved survival, the spectrum of pathologies causing abdominal pain in CF has shifted. Despite this, there have been relatively few previous publications focusing on gastrointestinal disease in CF adults. The aim of this review was to examine the characteristics, differential diagnosis, investigation, and optimal management of adults with CF presenting with abdominal pain.