The pooled results indicated that UGT1A1 rs3755319 (AC vs. AA, OR = 1.454, 95% CI: 1.100–1.921, P = 0.009), UGT2B7 rs7662029 (G vs. A, OR = 1.547, 95% CI: 1.249–1.917, P < 0.0001; GG + AG vs. AA, OR = 2.371, 95% CI: 1.779–3.160, P < 0.0001; AG vs. AA, OR = 2.686, 95% CI: 1.988–3.627, P < 0.0001), and UGT2B7 rs7439366 (C vs. T, OR = 0.585, 95% CI: 0.477–0.717, P < 0.0001; CC + TC vs. TT, OR = 0.347, 95% CI: 0.238–0.506, P < 0.0001; CC vs. TC + TT, OR = 0.675, 95% CI: 0.507–0.898, P = 0.007) might be associated with the risk of AT-DILI. The gene discussed is UGT1A1; the disease is ataxia telangiectasia.