An HIV infected hemophilia patient with huge inflammatory pseudotumor was in severe ill condition. The operation of right hip joint amputation was performed on the patient with perioperative infusion of coagulation factor VIII and highly active antiretroviral therapy (HAART). The pathological found that Inflammatory cell infiltration, formation of folliculus lymphaticus, muscular fiber breakage, and fibrous tissue hyperplasy, necrosis in both soft tissue and bone were seen in inflammatory pseudotumour. The vital signs remained stable during the operation and patient's overall health condition improved significantly ten months after operation. With the infusion of coagulation factor VIII and HAART, HIV infected hemophilia patient can be safety operation and may get effective result.