Abstract:Osteosarcoma is a kind of malignant bone tumor that often occurs in adolescents and has a strong metastatic ability and poor prognosis. The occurrence of osteosarcoma and resistance to cisplatin is known to be related to ERCC gene. As a key component of the nucleotide excision repair pathway, ERCC gene is found to regulate DNA repair. ERCC functional differences are mainly related to single nucleotide polymorphisms. The low expression may increase the risk of osteosarcoma, and the high expression may increase the resistance of patients to cisplatin. In addition, ERCC gene is of great significance to the incidence and prognosis of osteosarcoma and to guide the chemotherapy drugs selection. The targeted inhibition of ERCC provides possibility to reduce the resistance of cisplatin. Our review highlights the research progress of ERCC in osteosarcoma and provides relevant concepts for further clinical translational applications.