PurposeNimotuzumab is an IgG1 antibody that targets epidermal growth factor receptor (EGFR). Overexpression of EGFR is detected in some pediatric brain tumors including diffuse intrinsic pontine gliomas (DIPG)s.MethodsSince May 2010, nimotuzumab, combined with carboplatin or vinorelbine or Temozolomide (TMZ), was administered during progressive disease (PD) after the use of the institutional protocol consisting of radiotherapy (RT)+TMZ and adjuvant TMZ. After May 2012, children with newly diagnosed disease received TMZ during RT, and nimotuzumab and TMZ after RT. Nimotuzumab was given as 150mg/m(2)/dose once a week for 12weeks, and then every other week with TMZ until PD. PD patients were switched to nimotuzumab+vinorelbine combination until death.ResultsNimotuzumab was used in 24 children with DIPG (seven in the PD group, 17 in the newly diagnosed patient group). In the PD group, median survival time was 12months (7-42months); 1-year and 2-year overall survival (OS) rates were 42.918% and 14.3 +/- 13%, respectively. The median survival in this group, after the initiation of nimotuzumab was 6months (3-8months). In the newly diagnosed patient group, median survival time was 11months (3-35months) and median progression free survival was 4months (1-21months). The 1-year OS in this group was 35.3 +/- 11% and 2year OS was 11.8 +/- 7%. Nimotuzumab +/- chemotherapy was well tolerated with no major adverse effect.Conclusion Nimotuzumab-containing regimens are feasible and tolerable; it might be that some patients either with newly diagnosed DIPG or with progressive disease may benefit modestly from nimotuzumab-containing combinations.