Cardiac surgery for coronary heart disease, and for calcific and degenerative valvular heart disease, will likely become more frequent with an ageing haemophilia population. Our report describes the successful management of an individual with mild haemophilia B undergoing elective aortic valve replacement using a continuous infusion of recombinant factor IX. Emphasis is placed on the multidisciplinary coordination of care required across three hospital sites to ensure an uncomplicated peri- and postoperative course. We also provide a review of the current literature on cardiac surgery in patients with haemophilia B.