Creutzfeld-Jakob disease (CJD) is a rare disease but a common cause of rapidly progressive neurodegeneration. Although the “classic” presentation involves early dementia or behavioral changes, there are well-described atypical variants with less prominent cognitive symptoms at onset. One such variant is corticobasal syndrome (CBS), which may be seen with other underlying neurodegenerative processes; however when CBS is due to prion disease pathology, it typically involves a much more rapid progression and certain characteristic imaging findings. This report includes a case presenting with CBS without cognitive or behavioral changes at onset, which rapidly progressed and was determined ultimately to be due to underlying CJD. This case illustrates the need for a high index of suspicion for CJD to drive appropriate diagnostic testing and careful review of imaging and EEG findings, which may be subtle in early disease.