Background: The role of surgery in the management of gadolinium-enhancing pediatric brainstem lesions on magnetic resonance imaging (MRI) has been a matter of open debate. This clinical series correlates radiological and pathological findings to assess the role of contrast enhancement as an indication for surgery with respect to clinical outcome. Methods: We retrospectively reviewed the charts of all pediatric patients admitted to the Johns Hopkins Hospital with a diagnosis of a brainstem tumor between January 1985 and December 2000. Results: There were a total of 89 patients who met the inclusion criteria. Fifty-seven patients (64.0%) underwent surgical resection while 32 (36%) were treated with radiation and/or chemotherapy. Of the surgical candidates, 57 (100%) had an accompanying MRI scan significant for an enhancing lesion in the midbrain, pons or the medulla. The pathology was consistent with juvenile pilocytic astrocytoma in 30 patients (52.6%) and glioblastoma multiforme in 12 patients (21.1%). The remaining cases consisted of 10 patients (17.5%) with fibrillary astrocytomas, 3 (5.3%) with gangliogliomas, 1 (1.8%) with an oligodendroglioma and 1 (1.8%) with a primitive neuroectodermal tumor. A total of 29 patients had a total surgical resection, 8 a near total resection (>90%), 15 a subtotal resection (50–90%) and 5 a partial resection (<50%). The progression-free survival of all patients was 71.9% at 3 years and 45.6% at 5 years. Conclusions: This case series illustrates that contrast-enhanced MRI has positive prognostic value in the management of pediatric brainstem gliomas. In our study, the majority of enhancing tumors were low grade and amenable to surgical intervention. Consequently, we recommend surgical resection and pathological diagnosis of all enhancing brainstem tumors with adjuvant therapy reserved for recurrent or unresectable cases.