Brainstem vascular malformations can sometimes simulate other conditions such as multiple sclerosis and pontine glioma. We report a case of brainstem cavernous angioma for which clinical course and radiologic appearance were suggestive of a pontine glioma. The diagnosis was uncertain until the clinical and radiologic evolution made it clear that the patient had a cavernous angioma. We suggest that brainstem angioma be considered as a differential diagnosis for pontine glioma.