We report the clinical and EEG data of two patients harboring heterozygousSLC6A1mutations, who presented with typical absence seizures at 3 Hz spike and wave as well as with mild cognitive disability. Neuroradiological and other laboratory investigations were normal. Our observations suggest thatSLC6A1mutations can be suspected in children with typical absences as the only seizure type, especially if associated with, even mild, cognitive deficits.