A 6-year-old child with a brain stem tumor presented with the unusual complaint of intractable facial pain resembling trigeminal neuralgia in the absence of other symptoms or signs referable to the 5th cranial nerve. The radiological evaluation included a computed tomographic scan with intravenous contrast administration, which demonstrated an enhancing intramedullary lesion extending from the obex to C-4. After radiation and chemotherapy had failed to achieve symptomatic relief, the tumor, later proven to be a ganglioglioma, was radically removed with the ultrasonic aspirator. Postoperatively the patient experienced full pain relief.