This article reviews lung transplants for cystic fibrosis (CF) patients. Advances in medical therapy have resulted in increased survival for CF patients. Respiratory failure continues to be the leading cause of mortality. CF is a complex disease and a challenge for both CF and transplant physicians. This review covers the recent literature related to aspects of pre-lung-transplant mechanical ventilation and pre/post-transplant infection management. We also review post-operative management of the broad range of systemic complications related to cystic fibrosis, including diabetes, malnutrition, CF-liver disease, and malignancy. Improvements to surgical technique, organ procurement and preservation, and post-operative management in the intensive care unit could improve overall survival.