With increasing longevity, the prevalence of cardiovascular disease (CVD) risk factors in hemophilia patients is expected to increase; however, evidence-based guidelines on management are lacking. The aim of the study was to determine the prevalence and management of CVD risk factors in hemophilia patients. A retrospective study of 58 adult hemophilia patients (≥35 years) attending Mayo Comprehensive Hemophilia Center between 1 January 2006 and 15 October 2009 were reviewed. The prevalence of CVD risk factors was hypertension 65.5%, diabetes 10.3%, smoking 12.5% and obesity 19.6%. A total of 31% did not have a lipid profile on record. Management of risk factors included antihypertensive medications in 84.2% and lipid-lowering agents in 12.1%. During their medical evaluation, four of seven active smokers received smoking cessation counseling and four of 11 obese patients received lifestyle modification advice. Eight patients (13.8%) experienced a CVD event: myocardial infarction (MI) (n=3), coronary artery disease (n=2), both MI and ischemic stroke (n=1) and hemorrhagic strokes (n=2). Only five of eight patients were on low-dose aspirin, of which aspirin was discontinued in one patient after he was diagnosed with hemophilia following a bleeding work-up. Another patient on dual antiplatelet therapy post stent placement developed epistaxis resulting in clopidogrel cessation. Hemophilia patients are at risk for CVD, similar to the general age-matched male population. Screening for CVD risk factors, with preventive dietary and pharmacologic interventions, play a key role in the prevention and long-term management of CVD. Collaborative efforts between primary care providers, cardiologists and hemophilia center specialists remain essential in managing these complex patients.