The risk of infection in individuals with haemophilia using central vascular access devices for administration of clotting factor concentrates for prophylaxis or immune tolerance is unknown. We conducted a survey of US haemophilia treatment centres to determine the incidence and clinical characteristics of infection associated with the use of central venous catheters. Seventy (38.3%) of 183 patients using central lines developed device-associated infection, including 30 (28.0%) on prophylaxis and 40 (52.6%) on immune tolerance, P < 0.005. Over half (54.8%) the infections occurred in those 3 years of age. Implanted/tunnelled devices (port catheters) were more likely to become infected in the first 30 days after insertion, 11 of 41 (26.8%), than external catheters (broviac/hickman), none of 29 (0%), P= 0.00003. The median time to infection from initial device placement, 124 days, varied with age, 57 days in those 2 years of age vs. 161 days in those > 2 years of age, P= 0.0008, but not with type of device or treatment. Staphylococcal infections were more common with implanted devices (ports), 30 (73.2%), than external catheters, 12 (41.4%), P < 0.01, and Gram-negative infections were more common with external catheters, 17 (58.6%), than tunnelled devices, 7 (17.1%), P < 0.005. In summary, the rate of infection with central venous access devices in haemophiliacs is high, and alternative approaches to venous access should be explored.