Introduction. Cystic fibrosis (CF) is an inherited disorder that presents in childhood as a multisystem disease. Pulmonary failure and pancreatic insufficiency, including CF-related diabetes (CFRD) and exocrine insufficiency, are significant causes of morbidity and mortality in these patients. In this report we have reviewed our experience with a simultaneous lung and pancreas transplantation in a patient with CF. Methods. The recipient was a 25-year-old man with CF complicated by bronchiectasis with recurrent episodes of pneumonia, pancreatic exocrine insufficiency, and CFRD. He had normal hepatic and renal function. Surgical Technique. The lung and pancreas allografts were procured from a single cadaveric donor. The double lung transplantation was performed through separate thoracic incisions. The pancreas transplantation was performed through a midline incision with systemic venous drainage and proximal enteric exocrine drainage. Results. The recipient recovered well from his transplantation with early extubation. The pancreas allograft functioned well with normal blood glucose independent of insulin. As a result of the enteric drainage of the pancreas allograft, the patient no longer required supplemental pancreatic enzymes. His postoperative course was complicated by distal intestinal obstruction, a complex wound infection, and reversible leukoencephalopathy. At 1-year posttransplantation he remains free of supplemental oxygen, insulin, and pancreatic enzyme replacement. Conclusion. Simultaneous lung and pancreas transplantation in a patient with CF was performed safely, providing the advantages of normalization of glucose and improved nutrition for a patient requiring lung transplantation.