Patients with hemophilia are at high risk of bleeding following oral surgery. As an X-linked recessive chromosomal bleeding disorder it is very rare in female patients. This is the first described case of management of third molar removal in a female patient suffering from severe hemophilia B. Excellent hemostasis was achieved by following a protocol using defined pre- and postoperative doses of factor IX and local hemostatic measures of collagen fleece, fibrin glue, primary suture, and tranexamic acid solution. Following defined protocols is essential in the management of oral surgery in patients with hemophilia and helps to prevent postoperative hemorrhages. Keywords: bleeding disorder, factor replacement, female patient, hemophilia B, local measures, third molar removal