The patient history is the most crucial preoperative preparation tool for the management of hemophilia. Up to one-half of patients with severe hemophilia have a negative family history. There is no large-scale, prospective data correlating factor replacement to clinical outcomes in children with hemophilia undergoing surgery. Initial screening tests for a suspected congenital coagulopathy include a platelet count, prothrombin time (PT), and an activated partial thromboplastin time (aPTT). The recommended goal is personalized optimization of FVIII and FIX activity levels in the range 80–100 IU/dL (80–100% factor activity) before surgery and during active bleeding. Patients older than 2 years with mild hemophilia A and no inhibitors may be managed with intermittent doses of desmopressin (DDAVP). Adjuncts to coagulation factor replacement to minimize excessive bleeding in the perioperative period include antifibrinolytics, DDAVP, topical hemostatic agents, and plasma products when recombinant coagulation factors are unavailable. Global analysis of whole blood samples through techniques such as the thrombin generation assay or viscoelastic testing is becoming more accepted for acute bleeding management despite the absence of large, randomized trials or regulatory body approval. The US Centers for Disease Control (CDC) reports a significant improvement in mortality and hospitalization rates for patients managed through centralized hemophilia treatment centers (HTCs).