Hemophilia, the most common of the inherited bleeding disorder, is the result of a deficiency of clotting factor. Since bleeding after dental treatment may cause severe or even fatal complications, people with hemophilia must be given special dental care. We report on the diagnosis and treatment of a 9-year-old boy having severe hemophilia visited our department with the chief complaints of pus discharge on the left lower molar region. In the clinical and radiographic examination, periapical abscess and dental caries were diagnosed. Considering complexity of the treatment and complication in the coagulation, it was decided to carry on the treatment under general anesthesia. Clotting factor IX concentrates were intended to provide 50-70% plasma level. Pulpectomy, resin restoration and Stainless steel crown were given under general anesthesia. Several teeth were extracted and the sockets were packed with Surgicel (Oxidized Regenerated Cellulose, Johnson and Johnson Co. Neuchatel, Switzerland) under general anesthesia. Transpalatal arch and lingual arch were given for maintaining the extracted space before discharged. For people with severe hemophilia, factor replacement is necessary before scaling, surgery or regional block injections. Therefore, if several extractions are needed, dental care under general anesthesia would be effective and efficient management.