The early stage of sporadic Creutzfeldt-Jakob disease is generally characterised by progressive changes in behaviour and intellectual function. While only a few patients have stroke-like onset, Creutzfeldt-Jakob disease with initial monoparesis has been described. In this report, a patient with an unusual sporadic Creutzfeldt-Jakob disease with typical magnetic resonance imaging findings, positive cerebrospinal fluid 14-3-3 brain protein, sharp-wave complexes in electroencephalogram, and initial right hemiparesis is reported.