We describe 2 patients with acute demyelinating brainstem encephalitis who were treated with intravenous immunoglobulin (IVIG). Both patients improved rapidly, concomitant with the course of therapy. To the best of our knowledge, these are the first reported cases of brainstem demyelinating lesions in children treated with IVIG. Our experience suggests that IVIG should be considered as first-line treatment for similar cases.