Background: ABC proteins are one key type of transport superfamilies which undertake majority of drugtransport, which affect the osteosarcoma response to chemotherapeutics. Previous studies have suggested theassociation between ABC polymorphisms and osteosarcoma response. However, the results of previous studiesremain controversial. Therefore, we perform a meta-analysis to get a more precise estimation of this association.The association between ABC polymorphisms and osteosarcoma response was assessed by odds ratios (ORs)together with their 95% confidence intervals (CIs). Three polymorphisms of ABC including ABCB1 rs1128503,ABCC3 rs4148416 and ABCC2 rs717620 polymorphism were investigated. Overall, significant association wasobserved between ABCC3 rs4148416 polymorphism and osteosarcoma response under allele contrast (T vs.C: OR=1.73, 95%CI=1.09-2.74, P=0.019), homozygote comparison (TT vs. CC: OR=2.00, 95%CI=1.25-3.23,P=0.004), recessive genetic model (TT vs. TC/CC: OR=1.80, 95%CI=1.14-2.84, P=0.011) and dominant geneticmodel (TT/TC vs. CC: OR=1.70, 95%CI=1.20-2.42, P=0.003). Moreover, significant association was also observedin Caucasian population rather than Asian population for ABCB1 rs1128503 polymorphism. We conclude thatABCC3 rs4148416 polymorphism was significantly associated with poor osteosarcoma response and ABCB1rs1128503 polymorphism was significantly associated with good osteosarcoma response in Caucasian populationrather than Asian population.