A 58-year-old woman was admitted with left-sided decreased sensation, ataxia, and left "alien" hand and leg. Brain SPECT and PET showed hypoperfusion and hypometabolism in the right frontoparietal cortices, including the primary sensorimotor cortex, and temporal cortex. MRI demonstrated matching-restricted diffusion and fluid-attenuated inversion recovery hyperintensity. Corticobasal degeneration was suspected initially. The patient declined rapidly, and the diagnosis of sporadic Creutzfeldt-Jakob disease (CJD) was made based on cerebrospinal fluid biomarkers. CJD can present with symptoms similar to other disorders. This case illustrates that CJD can mimic a rare neurodegenerative disorder, cortico-basal degeneration, both clinically and by neuroimaging.