The association of CYP2R1 (rs1993116) with T1D susceptibility in Chinese children was confirmed in our case-control study, We found genotypes “CC” (OR = 2.968, 95% CI = 1.313–6.713, p = 0.009,) and “CT” (OR = 2.271, 95% CI = 1.066–4.838, p = 0.033,) in CYP2R1 (rs1993116) is significantly related to an increased T1D risk, Carriers of the CC, CT genotypes of rs1993116 have higher T1D risk than those carrying the TT genotype, and the C allele carriers of rs1993116 had a higher risk of T1D than the T allele carriers. Here, CYP2R1 is linked to type 1 diabetes mellitus.