The purpose of this study was to evaluate the association between X-ray repair cross-complementing group 1 (XRCC1) gene Arg399Gln and Arg194Trp polymorphisms and childhood acute lymphoblastic leukemia risk (ALL) risk. A systematic search of three databases was conducted. Odds ratios (ORs) with 95% confidence intervals (CIs) for XRCC1 polymorphisms and childhood ALL were calculated with fixed-effects models and random-effects models. This meta-analysis showed that Arg399Gln polymorphism was associated with increased risk of childhood ALL (Gln/Arg vs. Arg/Arg, OR = 1.25, 95% CI = 0.95-1.65, p = 0.032; Gln/Gln vs. Arg/Arg, OR = 1.44, 95% CI = 1.07-1.93, p = 0.448; dominant model, OR = 1.27, 95% CI = 0.98-1.66, p = 0.026; recessive model, OR = 1.16, 95% CI = 0.88-1.53, p = 0.646), while failing to detect links with the Arg194Trp polymorphism studied. In subgroup analyses, the pooled results showed that Arg399Gln polymorphism was associated with an increased risk of childhood ALL in Asians and larger sample size. However, no evidence of a significant association was observed in any subgroup of the Arg194Trp polymorphism. Our results provide evidence that the XRCC1 Arg399Gln polymorphism is associated with an increased risk of childhood ALL in the total population, especially Asians.