The increasing age of patients with congenital hemophilia poses new challenges for clinicians. Not only the consequences of bleeding but also age-related diseases, especially cardiovascular disorders, are of great concern. Factors such as hypertension, diabetes, or hyperlipidemia further increase the cardiovascular risk in elderly patients with hemophilia. Preventive treatment of these cardiovascular diseases may therefore also be necessary in hemophilia patients. However, determining the optimal level of anticoagulation in patients with reduced levels of coagulation factors is often difficult and requires an individualized approach. Given the absence of substantial evidence from large clinical trials, clinicians rely on case reports and expert opinions to guide the therapy. This case report details the individual risk-benefit assessment and management of a 57-year-old hemophilia A patient with atrial fibrillation.