Lung transplantation is indicated in certain patients with terminal pulmonary disease. We report a case in which a single lung (left) was transplanted to a 16-year-old girl with hemophilia B; she also suffered idiopathic pulmonary fibrosis and had a history of malnutrition, osteoporosis, severe scoliosis, hepatitis C positivity and recurrent bilateral pneumothorax. Treatment with pure factor IX was started the moment the donor lung was available and was continued for 37 days after surgery. Plasma levels of factor IX were kept at 100% during surgery and in the early postoperative period, and over 40% after that time. Correct hemostasis was thus achieved throughout the procedure, with no need for blood products. Patient outcome was satisfactory. The stay in the intensive care recovery ward was 17 days and discharge was 40 days after transplantation. We discuss aspects of hemophilia and lung transplantation, and the influence on malnutrition, chronic steroid treatment and osteoporosis.