To identify the association between UGT1A1 Gly71Arg and TATA promoter polymorphisms and neonatal hyperbilirubinemia.The studies related to the correlation between UGT1A1 Gly71Arg and TATA promoter polymorphisms and neonatal hyperbilirubinemia were searched systematically in various databases. According to the presence or absence of significant heterogeneity, a random-effect or fixed-effect model was chosen to estimate the overall odds rations (ORs) and 95% confidence intervals (CIs).Totally 21 studies on Gly71Arg polymorphism including 4738 neonates and 13 studies on TATA promoter polymorphism involving 2841 neonates were identified. Significant associations were presented between Gly71Arg polymorphism and neonatal hyperbilirubinemia in Asia [A vs. G, OR(95%CI): 2.327(1.904-2.845), P < 0.001; AA + GA vs. GG, OR(95%CI): 2.253(1.954-2.598), P < 0.001; AA vs. GG + GA, OR(95%CI): 5.166(3.520-7.564), P < 0.001; AA vs. GG, OR(95%CI): 6.458(4.376-9.531), P < 0.001; GA vs. GG, OR(95%CI): 1.920(1.654-2.228), P < 0.001] and Africa [A vs. G, OR(95% CI): 9.750(1.214-78.301), P = 0.032; AA + GA vs. GG, OR(95% CI): 11.000(1.290-93.832), P = 0.028; GA vs. GG, OR(95% CI): 10.000(1.163-85.998), P = 0.036]. TATA promoter polymorphism was associated with an increased risk of neonatal hyperbilirubinemia in Asia [TA7/7 vs. TA6/6 + TA6/7, OR(95%CI): 1.670(1.034-2.696), P = 0.036] and Europe [TA7/7 vs. TA6/6 + TA6/7, OR(95%CI): 2.627(1.722-4.008), P < 0.001].The risk of neonatal hyperbilirubinemia may be increased by the variation of UGT1A1 Gly71Arg in Asia and Africa, as well as the variation of UGT1A1 TATA promoter in Asia and Europe.