OBJECTIVES This study was designed to evaluate the efficacy and toxicity of the modified B-Non-Hodgkin's Lymphoma (NHL)-Berlin-Frankfurt-Münster (BFM)-90-based protocol in Chinese children and adolescents with Burkitt's lymphoma and large cell lymphoma. METHODS From September 1997 to August 2005, 55 untreated patients (age less than 20 yr) from a single institution were enrolled. The patients were stratified by risk factors (stage, LDH level and chemotherapy response). All patients were treated with a modified B-NHL-BFM 90 protocol. RESULTS The median age of the patients was 8 yr (range 1.5-20 yr). Of these patients, 22 (40%) had Burkitt's lymphoma (BKL), 22 (40%) had diffuse large B-cell lymphoma (DLBL) and 11 (20%) had anaplastic large T-cell lymphoma (ALCL). Complete remission (CR) occurred in 45 patients (83%), partial remission (PR) in eight patients (14.5%), and progressive disease (PD) in one patient (1.8%). At a median follow up of 24 months, the event free survival (EFS) for all patients was 85% +/- 5% with 100% for group R1, 84% +/- 7% for group R2 and 72% +/- 13% for group R3, and most notably, 80% +/- 6% for stage III/IV at diagnosis. There was no statistically significant difference (P = 0.96) in EFS among BKL and DLBL and ALCL. The major toxicity complications were myelosuppression and mucositis, but these conditions were tolerated and manageable. CONCLUSIONS This modified NHL-BFM-90 protocol is very effective for Chinese children and adolescents with BKL and large cell lymphomas, and represented an increase in the cure rates in childhood NHL in China.