Acute myeloid leukemia (AML) is a common type of acute leukemia characterized by the abnormal proliferation and accumulation of primitive and naive myeloid cells in the bone marrow and peripheral blood. Chemotherapy and hematopoietic stem cell transplantation (HSCT) are effective treatments for acute myeloid leukemia in hematology. However, some patients still experience relapse after achieving remission, including bone marrow relapse and extramedullary relapse. Extramedullary relapse of acute myeloid leukemia is more common in the central nervous system (CNS), skin, and soft tissue. The pathogenesis of extramedullary relapse is not fully defined and is generally believed to be related to abnormal cytogenetics, leukocytosis at diagnosis, male gender, total body irradiation (TBI), and the condition at the time of transplantation. Currently, the optimal treatment strategy for extramedullary recurrence is controversial. It is generally believed that locally performed combination therapies such as surgery, chemotherapy, and donor lymphocyte infusion are the best choice. Patients with extramedullary recurrence of acute myeloid leukemia have a poor prognosis and shorter median survival.