Brain stem gliomas constitute 10-20% of central nervous system tumors in children. Management of these tumors remains controversial. In general, the prognosis for long-term survival is poor, but brain stem gliomas represent a heterogeneous population of tumors, some of which have a better prognosis than others. Improvements in diagnostic imaging techniques, particularly the availability of magnetic resonance imaging, provide the means to differentiate among these populations. Advances in radiation therapy may provide the means to improve survival. In this article we review the reported series and the present state of the art for the diagnosis and management of children with brain stem gliomas.