Burkitt lymphoma (BL) is the most common lymphoma in childhood, which presented 30%-40% in non-Hodgkin lymphoma (NHL). In recent years, because of using the short course, high intensity chemotherapy, the prognoses of children with BL are improved obviously. However, some cases progress or recurr during treatment. The relapse/refractory cases are still seen in a few of children, and the prognoses are very poor for them. So far, there are no useful and efficiency protocol for these relapse/refractory cases. In order to find efficacy methods for children with relapse/refractory BL, this article summaries the domestic and foreign research progress on pathogenesis, recurrence related factors, drug resistance mechanisms, and treatment strategies for children with relapsed/refractory BL, finally to improve the prognosis for children with BL.