Hemophilia is a hereditary disease of blood clotting process with bleeding disorder. There are two main types, hemophilia A with the deficiency of factor VIII and hemophilia B with the deficiency of factor IX. The genes for factors VIII and IX are located on the X chromosome, therefore it is a recessive sex-linked disease, where women are carriers and men are affected. Family history is positive in two-thirds of patients. The clinical presentation largely depends on the level of coagulation factors and based on that there is a mild, moderate and severe hemophilia. The most common form of hemorrhage is bleeding in the joints or hemarthros. In severe hemophilia there are spontaneous bleedings, which may be fatal (bleedings in central nervous system, gastrointestinal system). In the diagnosis of hemophilia characteristic findings include prolonged aPTT, normal PT, normal bleeding time and normal platelet count. Confirmation of hemophilia A/B involves measuring the activity of factor VIII/IX. The treatment of acute bleeding should start as soon as possible with replacement therapy of missing factors. Prophylactic treatment includes the application of coagulation factors at regular intervals to prevent bleeding, and thus the prevention of chronic joint damage which is the most common complication of hemophilia. As a result of such treatment approach, today is the life expectancy of people with hemophilia similar to the general population. In very rare cases there is acquired hemophilia, with the development of severe hemorrhagic diathesis in people who previously did not have a bleeding tendency. Affects both men and women. It occurs due to the formation of autoantibodies to coagulation factors, mostly to factor VIII. The clinical presentation is dominated by extensive cutaneous and subcutaneous bleeding with possible fatal bleeding in internal organs. It is treated with immunosuppression in order to eradicate inhibitors to coagulation factors, and preparations of rFVIIa or APCC to stop active bleeding.