Creutzfeldt-Jakob disease (CJD) is characterized by the core symptoms of rapid progressive dementia, myoclonus, and typical periodic sharp wave complexes (PSWC) on electroencephalography (EEG). In recent years, imaging techniques have become more and more important in the diagnostic process of CJD. Magnetic resonance imaging (MRI) is very characteristic and the most accurate imaging technique to be of help in diagnosing CJD. In this study, we applied transcranial color-coded ultrasound (TCCS) in three cases of sporadic CJD. We found that all of them had in common a typical ultrasound tissue image of the lentiform nucleus, best to be described as a blurry inhomogeneous hyperechogenic signal pattern where usually the echogenicity is mostly homogeneously hypoechogenic.