We report serial MRI studies in a patient with Balò's concentric sclerosis, who presented with a progressive weakness in the right limbs of 10 day duration. Serial MR images revealed several concentric lesions characterized by layer by layer of demyelinated and myelinated appearance and multiple plaques in the white matter. Pathological study of a biopsy of a concentric lesion (which was performed to rule out neoplasm) further confirmed this diagnosis. These MR findings resolved in correspondence with clinical improvement. The patient was almost completely normal 1 year later. In conclusion, in this case, we found that MRI could show the striking feature reminiscent of the histopathologic findings of Balò's concentric sclerosis and played an important role in antemortem diagnosis of this rare demyelinating disease.