Gliomas that arise in the brain stem have been associated with a poor
prognosis. Diagnostic neuroimaging readily identifies the tumor as it
extends between normal brainstem structures. Histologic sampling of tu
mor with stereotactic methods is notoriously unreliable in establishin
g a definitive prognosis. Clinical trials that incorporate high-dose c
hemotherapy, autologous bone marrow rescue, and irradiation hold promi
se of better tumor control by overcoming the inaccessibility of the ce
ntral nervous system to standard doses of chemotherapy. We review the
pathology, clinical features, neuroimaging features, and current thera
peutic concepts relative to brainstem glioma. The pediatric neurologis
t has a pivotal role in identifying and monitoring children with this
malignancy.