We report a rare case of hyperglycemia-related osmotic demyelination syndrome (ODS) with focus on the imaging findings. A 61-year-old man with diabetes was admitted for general weakness and severe thirst. A few days later, he complained of dysarthria, dysphasia, and dysmetria. Laboratory examinations showed significant hyperglycemia, but normal electrolyte levels. Brain MRI revealed T2-signal abnormalities that were symmetrical, non-space occupying, and located in the central pons with a peripheral sparing pattern, which were suggestive of ODS. In addition, subsequent MRI revealed progression of signal hyperintensity; however, the patient’s symptoms improved.