The IKAROS family zinc finger 1 (IKZF1) gene encodes the Ikaros protein, which regulates the development and differentiation of hematopoietic cells, and is essential for autoimmune and tumor suppression.With the development of genomics, IKZF1 has become an important prognostic biomarker for the occurrence and progression of acute lymphoblastic leukemia.Mutations in the IKZF1 gene appears in about 15% of childhood acute B-lymphoblastic leukemia.The mutation impairs the tumor-suppressor function of the IKZF1 gene, enhances the proliferation and anti-apoptotic ability of leukemia cells, and develops resistance to key chemotherapy drugs.IKZF1 mutations are more common in patients with other adverse prognostic factors and face difficulties in the course of treament with high recurrence rate, short remission period and high mortality.Intensive therapy, hematopoietic stem cell transplantation and immunotherapy for childhood acute B-lymphoblastic leukemia with IKZF1 mutation can reduce the recurrence rate and improve the remission rate and survival rate.Targeted therapy is promising to improve the prognosis of children with IKZF1 mutation.