Lung transplantation has emerged as an acceptable option for the management of cystic fibrosis patients with endstage lung disease. Heart-lung transplantation and, more recently, double lung transplantation have been successfully performed in this group of patients. The choice of operation, so far, has been based on the surgeon's preference and experience as well as the cardiac function of the patient. Each of the procedures has advantages and disadvantages. This article reviews the current worldwide experience in lung transplantation for patients with cystic fibrosis and highlights the controversies involved in the selection of patients and procedure.