Objective　To explore the clinical and pathological features of large B-cell lymphoma with IRF4 gene rearrangement in children. Method　The clinical data of large B cell lymphoma with IRF4 gene rearrangement in 3 children were retrospective analyzed. Results　All the three cases were male and the age at onset was from 5 years and 4 months to 7 years and 10 months. The onset sites were tonsils, ileocecal, and cervical lymph nodes. The findings in histopathological morphology and immunohistochemistry of 3 children after surgery showed that the tumor cells were nodular or diffusely distributed. The tumor cells all expressed CD20, MUM1, BCL-6, and BCL-2, and they expressed CD10 in 2 cases. FISH was performed and showed fracture in IRF4 gene were negative in 2 cases and was positive in 1 case. All patients were diagnosed of large B-cell lymphoma with IRF4 gene rearrangement. All 3 cases were treated according to the CCCG-NHL-2016 regimen, 2 cases in group R2 and 1 case in group R3. Two patients had finished chemotherapy for more than one year, and no recurrence was found up to now. A child in R2 group with onset site of cervical lymph node was still under treatment and had achieved complete remission. Conclusion　Large B-cell lymphoma with IRF4 gene rearrangement in children is rare. FISH detection of IRF4 gene is helpful for diagnosis and its treatment can be according to the CCCG-NHL-2016 regimen.