Lung transplantation has recently offered hope for prolonged survival in patients with cystic fibrosis. Patients with cystic fibrosis have a 7% prevalence of associated liver disease and portal hypertension. These patients have been previously excluded from consideration for lung transplantation. The natural history of cystic fibrosis-associated liver disease suggests a benign and protracted course in most cases. At the University of Washington, 14 of 53 patients (26%) have undergone lung transplantation for cystic fibrosis-related respiratory failure. We report the outcome of double lung transplantation in four of these 14 patients who also had cystic fibrosis-associated liver disease and portal hypertension, all of whom were symptom free from their liver disease. All four patients are alive and well without complications 4 to 31 months after transplantation. We conclude that the presence of cystic fibrosis-associated liver disease with portal hypertension, in the setting of good synthetic function (albumin > 3.0 gm/L and normal prothrombin time), normal serum bilirubin, minimal varices, without ascites or encephalopathy, should not be an absolute contraindication to lung transplantation. We recommend that other transplantation centers also include this patient population in consideration for lung transplantation.