Hemophilic pseudotumors are a rare but serious complication of hemophilia. Surgical treatment of inflammatory pseudotumors in hemophilia with AIDS is associated with a high risk. Our review of the domestic and international literature has not yet reported surgical treatment of inflammatory pseudotumors in patients with hemophilia with AIDS. We recently treated a case of hemophilia with AIDS combined with a large inflammatory pseudotumor of the right lower extremity, and we report it as follows. The patient was diagnosed with hemophilia A at the age of 2 years due to traumatic bleeding, and since then, he has received several transfusions of coagulation factor VIII. In 2002, he was diagnosed with HIV infection and hepatitis B and C viral infections, and in May 2002, CD4 was measured at 70cell/uL, and highly active antiretroviral therapy (HAART) was administered: 3TC+D4T+EFV. On June 18, 2007, he was admitted to the Shanghai Public Health Clinical Center for Surgery with a right thigh mass and was diagnosed with 1. right thigh hematoma 2. AIDS 3. hemophilia A 4. hepatitis C 5. hepatitis B. The patient was admitted to the surgery department and consulted by several specialists in Shanghai, which concluded that the indication for surgery was clear and the risk of surgery was extremely high. The patient was given blood transfusions, albumin and other nutritional support, requiring 6,000 u of factor VIII, 1 human albumin, 2 u of washed red blood cells and 200 ml of plasma every other day, plus antiviral and antibiotic drugs, at a medical cost of more than 10,000 yuan per day. The hemoglobin was between 15-40g/L. Heart failure and renal failure occurred during this period, and symptomatic treatment and supportive therapy were given. After adequate preparation, the CD4 was retested and rose to 272cell/uL, hemoglobin was at 50.8g/L, and HIV-1 viral load was below the detection level. On July 28, 2009, a right hip arthrolysis was performed under general anesthesia. Intraoperative radial artery and central venous pressure monitoring, preoperative infusion of factor VIII of 2000 u one hour before surgery and continuous intraoperative infusion of factor VIII of 4000 u. Standardized sterile isolation procedures in the operating room prevented the risk of occupational exposure during surgery. After incising the skin of the patient’s right thigh root, the vascular ligation of the truncated surface was carefully separated and cut off to minimize surgical bleeding. After incising the right hip capsule, the femoral neck was loosely and brittlely fractured, and there was blood leakage from the femoral neck section, and the femoral head was carefully freed and removed from the acetabulum. Drainage was then placed and the stump was closed with sutures. Intraoperative monitoring of vital signs was always stable. The hemoglobin was 92g/L, and the general condition improved significantly. Hemophilia is a group of bleeding disorders with hereditary coagulation disorders, and bleeding symptoms are the main manifestation of this disease. Hemophilic arthropathy is mainly caused by repeated intra-articular hemorrhage, and if the epiphysis and epiphysis hemorrhage, it may lead to collapse and deformation of the epiphysis, resulting in aseptic necrosis, which is less common. Inflammatory pseudotumors in hemophilia are caused by repeated intra-articular or subchondral hemorrhages that lead to bone resorption or cystic changes. The bone marrow, joint capsule and articular cartilage are mechanically and chemically stimulated by fibrin, ferricyanin and other blood substances, resulting in necrosis and destruction. When the pressure in the bone is increased, it causes osteolytic or swelling destruction of the bone, and some of the extreme swelling can break through the bone cortex. Pseudotumors are often misdiagnosed as bone tumors because of the patient’s history of bleeding. Hemophilic pseudotumors have some of the X-ray signs of benign and malignant bone tumors, such as bone destruction, periosteal reaction and new bone formation. Therefore, detailed medical history and other examination data should be taken after consultation to make a correct diagnosis. In this case, the patient began to present with swelling of the thigh above the right knee joint, which was initially diagnosed as a hematoma, and after treatment with coagulation factor VIII, the hematoma not only did not absorb, but continued to increase in size. After it developed into a huge mass in the femur, the only way to remove the lesion was to amputate it. At first, the patient and his family did not agree to the amputation, but general supportive therapy could not control the development of the inflammatory pseudotumor. When the pseudotumor became highly distended and ruptured, large amounts of coagulation factor VIII, plasma, albumin, and red blood cells were required daily to maintain life. The patient’s consent for amputation was already combined with severe anemia, hypoproteinemia, and HIV, hepatitis C and B virus infection, making the surgery extremely risky. There are no national or international reports of surgery for giant inflammatory pseudotumors in AIDS hemophilia. The Shanghai Public Health Clinical Center is the designated hospital for the diagnosis and treatment of AIDS-related diseases and has the important responsibility of providing medical services for HIV-infected patients. In cases of AIDS hemophilia combined with giant inflammatory pseudotumor, hepatitis B and C, severe anemia, hypoproteinemia, and immunocompromised, another minor blow may be life-threatening. However, when surgical treatment is the only hope to get rid of the lesion, there is a high value in taking high risks to perform surgery. The key to surgery is to control bleeding. Because the inflammatory pseudotumor is so large and the upper edge is close to the root of the thigh, we have to dissect it close to the edge of the inflammatory pseudotumor in order to ensure that there is enough skin flap to wrap the stump during surgery. After the skin was gently cut with a scalpel, there was obvious exudate under the skin, and the exudate and subcutaneous tissue of the wound were pushed with suction while using a suction device to reveal every tiny blood vessel for accurate ligation or electrocoagulation to stop bleeding, and the femoral artery was freed and the femoral vein was double ligated and cut off. When the hip capsule was severed, the femoral head was necrotic, the femoral neck was fragile and fractured, and the femoral neck section continued to ooze blood, which made the operation more difficult. Careful freeing of the femoral head from the acetabulum. Thorough surgical trauma ligation or electrocoagulation was performed to stop the bleeding, and finally drainage was placed and the stump was sutured closed. The intraoperative bleeding was less than 300 ml. Thanks to the preoperative application of factor VIII 2000 u and the continuous intraoperative drip of factor VIII 4000 u, it was ensured that there was no significant blood leakage from the surgical wound and the coagulation function was basically the same as that of a normal patient. The postoperative period continued with the daily application of factor VIII 6000 u. The trabecular drainage tube drained about 300 ml of light red fluid on the same day. As the edema in the right buttock due to inflammatory pseudotumor subsided after surgery, the drainage tube gradually decreased daily and the drainage tube was removed 7 days after surgery. The stitches of the amputated stump were all removed 3 weeks after surgery, and the wound healed in one stage (see photo). The general condition of the patient improved significantly due to the removal of the lesion.