Creutzfeldt-Jakob disease (CJD) is a rare form of rapidly progressive neurodegenerative disorder. Literature survey reveals only four reported cases of CJD with epilepsia partialis continua. Here, we present a review and a case study, with video-EEG sequences and characteristic MRI findings, of a fifth patient who presented with epilepsia partialis continua in the very early stage of the disease and followed a fatal course which was faster than expected. [Published with video sequences].