The authors analyze the MEDLINE publications in the past 20 years on the treatment of diffusely growing brainstem tumors in children and adolescents. Radiation and chemoradiation are main treatments for these diseases. The use of different radiation dose fractionation modes and different combinations of cytotoxic drugs has caused no significant changes in long-term results. Whatever the used treatment option is, most patients die within the next 1-2 years after tumor progression. Prognosis mainly depends on the biological properties of a tumor and patients with low-grade glioma generally survive longer. The late effects of radiotherapy make quality of life worse in long-term survivors after treatment. Hopes to enhance therapeutic effectiveness are placed on the development of targeted therapy options based on molecular biological markers.