We present a 45 years old man with neuroacanthocytosis. This gentleman has complex partial seizures and generalized tonic-clonic seizures, as well as movement disorders characterized by chorea and orofacial diskinesia. Complementary examination shows acanthocytosis of 11% on peripheral blood, irritative focus on right temporal lobe on EEG, serum creatinokinase of 101 U/l and volume reduction and hypersignal on caudate nucleus and putamen bilaterally on MRI.