Several studies have been conducted to examine the association between IKZF1 rs4132601 polymorphism and acute lymphoblastic leukemia (ALL) risk. However, the conclusions remain controversial. We therefore performed a meta-analysis. PubMed, Embase, Web of Science, Weipu and Chinese Biomedical Literature (CBM) databases were searched. Odds ratios (ORs) with 95% confidence intervals (CIs) were used to assess the strength of association. A total of 15 case-control studies with 8333 cases and 36 036 controls were included in this meta-analysis. The results suggested that rs4132601 was associated with an increased ALL risk. Significant associations were found among Caucasians and Hispanics but not among Asians. In subgroup analysis by age group, both adults and children showed increased ALL risk. In subgroup analysis by subtype of ALL, significantly increased risks were observed in B-cell ALL and B hyperdiploid ALL, but not in T-cell ALL. This study suggests that IKZF1 rs4132601 polymorphism is a risk factor for ALL.