Purpose: Paroxysmal non-kinesigenic dyskinesia (PNKD) should be included in the list of differential diagnosis in patients with refractory epilepsy. Although the pathophysiological mechanisms that underlie this disorder remain controversial, it is now accepted that the basal ganglia are the anatomical substrate responsible for it.Material and methods: We report a 16-year-old mentally retarded male with PNKD admitted for video-EEG monitoring and ictal SPECT, which showed hyperperfusion on the right caudate and thalamus.Conclusion: This case supports more evidence for the involvement of the caudate nucleus and thalamus in the mechanisms responsible for the production of PNKD.