Elective surgical procedures involving central venous access devices (CVADs) in patients with haemophilia are often necessary for adequate factor delivery but there are few data regarding haemostatic coverage and acute complication rates accompanying these procedures. To describe experience with CVAD insertion, revision and removal in young haemophilia patients at our institution and in the literature and to assess acute complications following CVAD procedures. PubMed, Medline and Cochrane databases were searched for articles, which included a description of factor coverage during CVAD procedures. A retrospective review of our comprehensive haemophilia database identified patients undergoing CVAD placement, revision and removal between January 1993 and August 2005. Manual and electronic searches of the published literature yielded 14 articles, which met inclusion criteria. Peri-operative factor administration varied greatly among the reports. Mean acute infection and haematoma rates were 8% and 12.5% respectively. A retrospective review identified 49 CVAD placements, revisions, or removals meeting inclusion criteria. Most patients received outpatient bolus factor replacement to achieve a level of 100% preoperatively, immediately postoperatively and on postoperative days 1, 2, 3, 5 and 7. Thirty-six procedures were performed without hospitalization. Ten patients developed 11 (22%) minor haematomas postoperatively. Major haemorrhage, acute infection, or pneumothorax was not encountered. Few published data exist regarding haemostatic coverage and complications following CVAD procedures. Our institutional experience using a consistent management approach was favourable. Further studies are required to define optimal haemostatic coverage during minor surgical procedures in haemophilia.