A 36 year old lady with possible sporadic Creutzfeldt-Jakob disease which manifested with rapidly progressive cognitive decline and abnormal behaviour, pyramidal, extrapyramidal signs and akinetic mutism. Scalp EEG findings show diffuse slowing of background activity of delta range. MRI brain revealed high signal intensity in bilateral caudate nuclei and putamen on T2WI, FLAIR sequence and DWI. The role of MRI in diagnosis of the CJD is discussed.