Out of 27 children with intracranial cavernous angiomas observed in a 17-year period of time, 3 presented with their lesion located in the brain stem. A further 19 pediatric cases of brain stem cavernous angiomas sufficiently well described for a reliable analysis were collected from the literature and evaluated for the present study. The widespread use of MR imaging has significantly increased the possibility of recognizing brain stem cavernomas at an early age; indeed, their incidence is nowadays regarded to range between 9 and 15% of pediatric cavernous angiomas. A female predominance was noted in the present study. Focal neurological deficits are the most frequent presenting symptoms; they tend to occur acutely in most cases, although subsequent improvement may then be observed in several patients. Recurrent hemorrhages from the angioma result usually in heavier neurological signs. Surgical approaches have to be planned according to the location of the lesion, and to the site where the angioma is eventually in contact with the pial or ependymal surface. No surgical deaths were observed in these 22 children; only 2 patients presented a mild persistent worsening of their neurological status after the operation. These good results indicate that brain stem cavernous angiomas are actually a surgical lesion in spite of their apparently unfavorable location. The good prognosis is related to the anatomophysiological features of these lesions, which usually displace the brain stem structures rather than invade them, and are characterized by a low-pressure and slow blood flow circle.