Abstract Background The role of neuroimaging in dementia extends beyond its traditional role of excluding neurosurgical lesions. Radiological findings may support the diagnosis of specific neurodegenerative disorders and aid differentiation from other neurodegenerative conditions e.g. CJD1. sCJD is a fatal neurodegenerative disease, which typically presents with neuropsychiatric symptoms, myoclonus, cerebellar and pyramidal/extrapyramidal symptoms. The diagnosis often is quite difficult, requiring high degree of suspicion, advanced neuroimaging and biochemical markers. Confirmation is only possible via brain biopsy. The condition can result in rapidly progressive dementia/neurocognitive decline and death, usually within a year from onset. Methods We report a case of a rapidly progressive dementia in a 79-year-old male who was diagnosed with probable sCJD, after having an MRI which picked up suspicious characteristic findings leading to the eventual diagnosis confirmation via further workup. The patient presented with acute rapid progressive forgetfulness and functional decline of 2 weeks with initial workup unyielding. Results A contrast MRI Brain eventually showed focal increased T2 and flair signals in the right parafalcine and parietal cortex demonstrating mild fluid restriction without any focal contrast enhancement, a pattern highly suspicious for sCJD. A CSF sample subsequently revealed RT-QulC positivity confirming the diagnosis. The patient eventually passed away despite ward-based care after 4 weeks of admission due to rapid neurological decline. A brain biopsy was not deemed feasible given patient’s condition. Conclusion The case highlights a relatively atypical symptomology of sCJD and, highlights the role of MRI imaging in assessment and diagnosis of dementias and specifically in sCJD. Increasing use of advanced neuroimaging may aid in diagnosis of specific/rare causes of rapidly progressive dementias. Reference Vitali, P., Maccagnano, E., Caverzasi, E., Henry, R.G., Haman, A., Torres-Chae, C., Johnson, D.Y., Miller, B.L. and Geschwind, M.D., 2011. Diffusion-weighted MRI hyperintensity patterns differentiate CJD from other rapid dementias. Neurology, 76(20), pp. 1711-1719.