The knee joint is one of the most commonly involved joints in hemophilia, accounting for almost half of the cases. As a result of repeated bleeding leading to joint destruction, patients successively develop disability in middle and young adulthood, with a progressive decline in quality of life and eventual severe incapacitation. Intra-articular hemorrhage is the most common and disabling clinical manifestation of hemophilia and occurs in patients with severe hemophilia. At the end stage of the disease, pigmentation and hyperplastic synovial tissues become fibrous, leading to joint contracture and eventually to fibrous ankylosis. Extensive joint surface erosion and destruction and severe limitation of motion cause pain, loss of life and impaired function. These are all surgical indications for total knee arthroplasty in patients with hemophilia. Because coagulation disorders are well controlled by coagulation factor concentrates, total knee arthroplasty is an alternative to knee fusion for the treatment of chronic hemophilic knee disease. After total knee arthroplasty, the patient’s pain disappears or is reduced, joint mobility improves, and work and life standards are enhanced. For patients with hemophilia, adjustment of preoperative, intraoperative and postoperative levels of coagulation factors is key to the procedure, and an experienced hematologist can be of great help to the orthopedic surgeon. Through preoperative laboratory tests, including routine blood work, coagulation factor analysis, and circulating antibody testing, the hematologist develops a detailed coagulation factor supplementation protocol and adjusts the amount of coagulation factor supplementation based on postoperative and intra-articular bleeding that occurs during functional joint exercise. The cost of treatment for hemophiliacs is dominated by the cost of western drugs, mainly coagulation factors, which can reach 48.2%. At the same time, some of the affected limbs are involved in both knees, and for the treatment of these patients, bilateral knee replacements can be completed in a single operation if conditions permit, thus saving the patient’s medical expenses. Since more severe joint deformities, soft tissue contractures or bone defects are often encountered during surgery, surgeons with extensive experience in replacement surgery perform the surgery for hemophiliacs.