A 44-year-old woman was referred to our hospital for massive subcutaneous and intramuscular hemorrhage. Prolonged APTT, low factor VIII activity and factor VIII inhibitor with high titer (30 BU/ml) were observed, confirming the diagnosis of acquired factor VIII inhibitor. Although treated with methylprednisolone, she relapsed after a month. Subsequently, she was treated with three courses of CVP (cyclophosphamide, vincristine, prednisolone) therapy, combined with recombinant activated factor VII. The activity of factor VIII was normalized one week after starting CVP, and the inhibitor disappeared 13 months later. She has maintained complete remission for 26 months without recurrence to date. CVP therapy is very effective against refractory acquired factor VIII inhibitor.