Creutzfeldt-Jakob disease (CJD) is a neurodegenerative disorder belonging to the group of transmissible spongiform encephalopathies. The transition of physiological, soluble, neuroprotective prion protein PrPc into its insoluble, misfolded isoform PrPSc remains its central pathogenic event. The progressive accumulation of isoform PrPSc within the brain tissue results in spongiform degeneration and a plethora of clinical symptoms. Typically, CJD manifests as progressive dementia with myoclonus, visual or cerebellar dysfunction, pyramidal/extrapyramidal signs or akinetic mutism. However, a growing number of studies indicate that CJD may present with prodromal psychiatric manifestations including anhedonia, anxiety, irritability, depression, insomnia, psychosis and catatonic behavior. We present a case of CJD with a history of prodromal psychiatric symptoms and catatonic behavior diagnosed by brain biopsy.