A 21-year-old man was troubled with a subacute history of brainstem involvement and a leukocyte pleocytosis in the cerebrospinal fluid. Magnetic resonance imaging (MRI) demonstrated a massive lesion with Gd enhancement in the pons. Steroid therapy, not antiviral drugs, was dramatically effective for the mass reduction and symptom improvement. Over 4 years no recurrence has been recognized, so this case was diagnosed to be an unusual case of acute disseminated encephalomyelitis (ADEM). ADEM must be included in the differential diagnosis for a brainstem mass in MRI.