Abstract: Objective: To evaluate the effect of custom-made artificial total knee prosthesis replacement on pathological fracture of distal femur caused by giant cell tumor of bone. Methods:A total of 12knees of 12 patients with pathological fracture of distal femur caused by giant cell tumor of bone were treated with cus-tom-made artificial total knee prosthesis replacement. There were 5 males and 7 females aged from 23to 40 years (34.2 years on average). Results: All patients tolerated surgery very well. The surgical duration was 120 ～180 min and the intraoperative bleeding was500 ～900 mL. The drainage tube was taken out in5 days after surgery. The postoperative drainage volume was 300 ～700 mL. There were no postoperative complica-tions. The postoperative X-ray examination showed that all of the prosthesises were in the right position. Lo -cal pain disappeared in all patients after surgery. The pathological examination identified all the patients with giant cell tumor of bone. All patients were followed up for 18～30months ( 24months on average). No recur-rence was observed after surgery. No infection or implant loosening occurred during the follow up. According to the Enneking assessment system of function after limb salvage surgery, the mean Enneking scale was 23.8 points (ranged from 15to 28points) at the last follow-up visit. Conclusion:Custom-made artificial total knee prosthesis replacement can achieve a high satisfactory rate in clearance of lesion and restoring function of the knee in patients with pathological fracture of distal femur caused by giant cell tumor of bone, improving patients’quality of life.