Aicardi-Goutières syndrome is characterized by the calcification of basal ganglia, leukodystrophy, and lymphocytosis in cerebrospinal fluid. No brainstem lesion has been described. We report a Japanese girl who presented with delayed development and microcephalus at early infancy. Magnetic resonance imaging revealed T2-weighted high intensity in the cerebral white matter and brainstem, and nerve conduction velocity was delayed in the central nervous system, indicating that she manifested dysmyelination in the brainstem white matter similar to that in the cerebral white matter.