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Cystic fibrosis (CF) is an autosomal recessive genetic syndrome that results in a mucoviscidosis that affects the lungs, pancreas, and other systems in the body and remains the most common lethal genetic disease in the Caucasian population . Modern prenatal screening has allowed for earlier identification, diagnosis and management of patients with CF, and substantial advancements in both the medical and surgical care of individuals with CF has resulted in improved long term survival. Much of this progress can be attributed to a combination of the development of comprehensive CF centers and a more thorough understanding of the pathophysiology of CF. Mutations in the CF transmembrane (conductance) regulator (CFTR) gene result in CF. The alteration in CFTR results in an abnormal electrolyte content in the environment external to the apical surface of epithelial membranes. This leads to desiccation and reduced clearance of secretions from tubular structures lined by affected epithelia. Therapeutic strategies including airway clearance, inhaled medications, modern antibiotic therapy, and pancreatic enzyme replacement therapy are aimed at managing the sequelae of the mucoviscidosis. This understanding of the pathophysiology of CF combined with modern anesthesia techniques has led to improved surgical outcomes in these patients. Pediatric surgeons must remain up to date on the surgical implications of CF, and the current advancements in medical and nutritional care.