Objective To review the methods of repair and reconstruction of the large segmental bone tumor defect in distal tibia. Methods The related literature of repair and reconstruction of the large segmental bone tumor defect in disatal tibia was reviewed and analyzed in terms of the reserved ankle joint and the non-reserved ankle joint. Results For large segmental bone tumor defect in distal tibia, the conventional allograft bone transplantation, vascularized autologous fibular transplantation, vascularized fibular allograft, inactivated tumor regeneration, distraction osteogenesis, and bone transport techniques can be selected, and the membrane-induced osteogenesis, artificial tumor stem prosthesis, three-dimensional printed metal trabecular prosthesis, ankle arthrodesis, artificial tumor ankle joint placement surgery are gradually to be applied to repair the bone defect. Moreover, due to its long survival time, the function of reconstructed bone tumor defect in the distal tibia has also received increasing attention. Conclusion Although the ideal methods has not yet been developed, great progress has been achieved in repair and reconstruction of the large segmental bone tumor defect in the distal tibia. Recently, with the appearance of three-dimensional printing and various preoperative simulation techniques, personalized and precise therapy could become ture, but therapies for the large segmental bone tumor defect in the distal tibia still need to be further explored.