Based on six autopsy and fifteen biopsy materials gross and microscopic findings of tuberous sclerosis (Bourneville’s disease) are presented and discussed along with the descriptions already available in the literature. Immature or dysgenetic Schwann’s cells appear to be one of the basic abnormalities in this syndrome, particularly in the kidneys, the skin and the central nervous system. Neoplastic changes in the kidney lesions and from the subependymal nodules of the brain are discussed together with the literature review on this subject. Histological features that would help to differentiate between simple hamartomas and true neoplasms are listed.