Twenty-three cases of tuberous sclerosis, found in the ten years' material of the authors among 2900 epileptics between o-16 years of age, are presented. 11 patients were treated before the age of 3 years, all showing infantile spasms. The earliest, in most cases congenital extraneural signs were depigmented areas on the skin. They were absent only in one case. They allowed, together with the Westsyndrome to establish the diagnosis if tuberous sclerosis before the appearance of other characteristic symptoms. Sebaceous adenoma, generally accepted as pathognostic for tuberous sclerosis, presented itself in all 12 early cases later then the leukoderma and the epileptic fits. Tuberous sclerosis is one of the important causes of epilepsy in childhood, primarily in early childhood. In this period of life the West-syndrome is the most frequent type of epileptic manifestations of tuberous sclerosis