A 27-year-old man with CF complicated by moderately severe suppurative lung disease, biliary cirrhosis, pancreatic exocrine insufficiency, and diabetes presented on three separate occasions with massive hemoptysis between 2007 and 2008. He had advanced liver disease with severe portal hypertension and previous variceal bleeds. He experienced chronic hemoptysis, particularly during periods of pulmonary exacerbations or high-intensity exercise. In October 2007, he presented with massive hemoptysis, expectorating > 500 mL of blood during the 6 hours prior to presentation. Hemodynamics revealed a systolic BP of 100 mm Hg and a pulse rate of 110 beats/min. He was given packed RBCs, fresh-frozen plasma, and platelet transfusions as needed to reverse his coagulopathy and thrombocytopenia. He was started on IV ticarcillin/clavulanic acid and tobramycin. Urgent BAE identified an enlarged left upper lobe bronchial circulation, which was embolized. The right bronchial circulation was not identified radiologically despite rigorous searching. Four hours postembolization, he experienced hemoptysis, expectorating a further 500 mL of fresh blood. He was given a single dose of rFVIIa, 90 μg/kg. Bleeding slowed, but the patient continued to have ongoing hemoptysis with approximate volumes of 100 mL/d over the next 3 days. On hospital day 5, a repeat BAE was performed. A slightly enlarged right bronchial artery that was not detected on previous examination was embolized. The repeat BAE achieved complete hemostasis with no further bleeding.