Susac syndrome (SS) is a clinical triad of hearing loss, retinal artery occlusion and encephalopathy. The typical MR imaging findings of multiple focal lesions in the corpus callosum and subcortical white matter can be easily misdiagnosed as multiple sclerosis. On diffusion-weighted (DW) MR imaging, new lesions were hyperintense, with reduced apparent diffusion coefficient (ADC). These lesions later became less prominent or hypointense on subsequent DW MR imaging. Serial DW imaging and ADC maps may be useful in differentiating SS from demyelinating diseases.