Lung transplantation (LT) under urgency-code mechanical ventilation (UCMV) has been identified in the International Society for Heart and Lung Transplantation (ISHLT) Registry as a negative prognostic factor increasing the likelihood of mortality. The objective of this study was to review our experience of UCLT for with cystic fibrosis (CF) patients compared with elective LT (ELT). From October 1993 to October 2007, we performed 259 consecutive LTs in 250 patients, of whom 78 (31.20%) had CF. Our study group comprised CF patients who received UCLT (n = 23). The type of LT in the UCLT group was as follows: bipulmonary (18), left unipulmonary (2), and bilobar transplantation from cadavers (3). The UCLT group more often required cardiopulmonary bypass (CB) (P = .025), pulmonary tailoring (P = .030), and longer periods of pulmonary ischemia (P = .066) than the ELT group. We noticed a greater number of cases of pneumonia during the first postoperative month in the UCLT group. However, incidence of surgical complications, early and perioperative mortality, and episodes of acute and chronic rejection (bronchiolitis obliterans syndrome) did not differ between the groups. Survival rates at 1, 3, 5, and 10 years were 73.66%, 63.74%, 42.49%, and 42.49%, respectively, in the UCLT group (mean, 1927 [SE = 366] days) and 75.95%, 71.32%, 63.37%, and 63.37% in the ELT group (mean, 2946 [SE = 281] days; P = .3417). In our experience, UCLT in patients with CF is fully justified. Careful selection of such cases permits acceptable long-term survival rates to be achieved with no increase in early or perioperative mortality.