A 26-year-old man with osteogenesis imperfecta and severe aortic regurgitation was scheduled for aortic valve replacement. As previously described by other authors the operation was difficult owing to the friability and weakness of the tissues. Mean blood losses of 153 mL per hour during the first 7 postoperative hours were observed. Despite normal coagulation indicators the bleeding did not stop and recombinant factor VIIa was applied at 40 microg/kg. Bleeding was successfully stopped after this single application.