During three decades, the demographics of cystic fibrosis (CF) has undergone a significant change. Advances in nutritional and pulmonary management allow the vast majority of patients reaching adulthood today. With increasing survival, new and previously less common aspects of CF are encountered by the clinician expanding the concept of CF as a multisystem disease. The first part of this two‐part review will focus on the nutritional and gastrointestinal aspects of the CF phenotype and outline core principles of diagnosis and care.