Wilms' tumor is the most common childhood renal malignancy. A genome-wide association study identified LIM domain only 1 (LMO1) as having oncogenic potential. We examined the associations between LMO1 gene polymorphisms and susceptibility to Wilms' tumor. In this hospital-based, case-control study, we recruited 145 children with Wilms' tumor and 531 cancer-free children. Four polymorphisms (rs110419 A>G, rs4758051 G>A, rs10840002 A>G and rs204938 A>G) were genotyped using Taqman methodology. Odds ratios (ORs) and 95% confidence intervals (CIs) were calculated to measure the associations between selected polymorphisms and Wilms' tumor susceptibility. Only rs110419 AG was found to be protective against Wilms' tumor (adjusted OR = 0.62, 95% CI = 0.41-0.94, P = 0.024) when compared to rs110419 AA. Wilms' tumor risk was markedly greater in children with 1-4 risk genotypes (nucleotide alterations) than in those with no risk genotypes (adjusted OR = 1.84, 95% CI = 1.25-2.69, P = 0.002). In a stratified analysis, the protective effect of rs110419 AG/GG was predominant in males. The association of 1-4 risk genotypes with Wilms' tumor risk was limited to subgroups of children who were >18 months old, female, and in clinical stages III+IV. Thus, LMO1 gene polymorphisms may contribute to Wilms' tumor risk, but this conclusion should be validated in other populations and larger studies.