The case is reported of meningioangiomatosis of the brain stem in a 3 1/2-year-old girl who suffered from vomiting, left facial weakness, difficulty in swallowing, and ataxia. This is believed to be the first reported case of meningioangiomatosis in the brain stem. Computerized tomography showed an intensely enhancing hyperdense mass in the left restiform body. Magnetic resonance imaging revealed that the lesion was isointense to gray matter on the T1-weighted image and hypointense on the T2-weighted image, with a surrounding zone of high T2 signal and intense enhancement. Angiography was normal. Surgical exploration demonstrated an intramedullary firm mass that was partially resected. Histologically, the mass consisted of a low-grade lesion of meningeal origin with spindle cells in a whorling pattern that were occasionally focused around small vessels. On 2-year follow-up imaging, the lesion remains unchanged in size. Certain particularities of this lesion are discussed in the context of the literature.