Primary metastatic diffuse intrinsic pontine glioma (DIPG) is relatively rare 
and associated with a dismal prognosis. Combining craniospinal irradiation (CSI) 
with concurrent temozolomide and nimotuzumab therapy may slightly improve tumor 
control and overall survival. However, little is known about the feasibility and 
toxicity of this treatment approach. Here, we describe the case of an 8-year-old 
girl with primary metastatic DIPG who received craniospinal radiotherapy, a 
local boost, and concurrent temozolomide and nimotuzumab treatment based on an 
individual therapy recommendation. Radiotherapy could be completed without any 
interruption. However, concurrent temozolomide had to be disrupted several times 
due to considerable acute myelotoxicity (grade III-IV).Maintenance 
immunochemotherapy could be started with a delay of 5 days and was performed 
according to treatment schedule. The disease could be stabilized for a few 
months. A routine MRI scan finally depicted disease progression 5.7 months after 
the start of irradiation. The patient died 1.9 months later.