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BACKGROUND: Creutzfeldt-Jakob disease is the prototype of prion illness in humans, it is a rare and clinically heterogeneous entity as well as an important differential diagnosis in rapidly progressing dementias. The definitive diagnosis is based on a biopsy of brain tissue; however, given the limitations of this study, there are diagnostic test that, in combination with the clinic, make a probable diagnosis. In Mexico there are few registered cases and there are no epidemiological data in this regard. CLINICAL CASE: A 69-year-old male patient from Nuevo Leon, Mexico, without a significant personal history who started with a rapidly progressive dementia and myoclonic movements who presented a 14-3-3 positive protein in cerebrospinal fluid, the electroencephalogram and nuclear magnetic resonance both with typical data for Creutzfeldt-Jakob disease. CONCLUSIONS: Creutzfeldt-Jakob disease is little suspected and in Mexico is little reported; before this situations it is important to have it in mind and include it among the differential diagnoses of neurological disease. [ABSTRACT FROM AUTHOR]