We report the case of a 36-year-old hemophilia B who suffered from cholesteatoma and underwent tympanoplasty. Though the factor IX activity was less than 1% of normal before surgery, adequate replacement of factor IX led to the achievement of hemostasis during and after surgery. The cholesteatoma was completely extirpated with matrix, and a type I canal-up tympanoplasty was subsequently performed. Careful preoperative evaluation and close cooperation with the hematologist are required if surgery is to be successful. We also present here the use of continuous administration of monoclonal antibody-purified factor IX concentrate, Christmassin M.