Objective　To explore the inducement and possible mechanism of spontaneous remission in acute myeloid leukemia. Method　The clinical data of acute monoblastic leukemia in a child with spontaneous remission were reviewed, and the possible mechanism of spontaneous remission was discussed in combination with relevant literature. Results　A year and 4 months old boy was diagnosed with acute monoblastic leukemia and had a spontaneous remission without chemotherapy. About 1 year later, the boy relapsed with myeloid sarcoma and received HLA haploidentical hematopoietic stem cell transplantation. The prognosis was good. Previous studies showed that most patients with spontaneous remission had a history of fever that was usually caused by infection, but could also be caused by drug withdrawal or blood transfusion. Conclusion　Spontaneous remission of acute myeloid leukemia is extremely rare. Immunotherapy may be employed to treat leukemia in the future.