Abstract: Objective　To investigate characteristics and clinical significance of IG/TCR rearrangement in children with acute lymphoblastic leukemia (ALL). Methods　Bone marrow samples and clinical data of 189 children with ALL were collected, and the IG/TCR rearrangements of patients were detected by multiplex polymerase chain reaction according to the method provided by BIOMED-2. Results　In our study, 176 children with ALL had IG/TCR rearrangement (102 male and 74 female). 136 cases (77.3%) has IGVH rearrangement and 46 ALL patients (26.1%) has a IGDH rearrangement. IGK rearrangement was detected in 92 patients (52.3%), and IGλ was detected in 11 patients (6.3%). 47 cases (26.7%) has TCRB rearrangement, and 77 cases (43.8%) have TCRD rearrangement. TCRG rearrangement was detected in 88 patients (50.0%). About 25 patients have only one clone of the IG/TCR rearrangement, about 41 patients have two clones of IG/TCR rearrangement, 61 patients have three clones, and 49 patients have more than three clones. We found IGK positive rate was higher in patients with high risk leukemia than that of medium risk and standard risk group , and the difference was statistically significant ( χ2=7.475， P=0.024), and other IG/TCR rearrangement has no association with risk degree in ALL patients (P>0.05). Conclusions　The rearrangement of IG/TCR in children with ALL is ubiquitous, and the IGK rearrangement is associated with the risk of ALL in children.