Eighteen of 418 children who had onset of epilepsy before the age of 13 years showed clinical and electroencephalographic evidence of benign childhood epilepsy with occipital paroxysms. They represented one-fifth of all benign age- and localization-related idiopathic epilepsies seen. Some patients were followed as long as 15 years. There was a preponderance in females and peak age at onset of epilepsy was 5 years. In 16 children, the seizures were infrequent and sometimes prolonged and consisted mainly of tonic deviation of the eyes and vomiting, often with evolution to unilateral or generalized convulsions. Seizures were only nocturnal in 11 and nocturnal and diurnal in another 5 children. Prognosis was excellent; 5 children had only one fit. Remission usually occurred 1 to 2 years after onset and no seizures occurred after the age of 12 years. The remaining 2 children had frequent diurnal episodes consisting of visual hallucinations, postictal headache, and occasional nocturnal hemiconvulsions. Their prognosis was less favorable. Electroencephalographic abnormalities in all 18 patients consisted of repetitive spike and slow-wave discharges confined to the occipital regions and attenuated when the eyes were open. These outlasted clinical remission for many years, sometimes up to the age of 16. Fixation-off sensitivity was demonstrated frequently. Based on these findings, a unifying definition for benign childhood epilepsy with occipital paroxysms is proposed.