Tuberous sclerosis (TSC) is an autosomal dominant disease arising from genetically programmed hyperplasia of ectodermal and mesodermal cells and manifested by a variety of lesions in the skin, central nervous system, heart, kidney, and other organs. The principal early clinical manifestations are the triad of: seizures, mental retardation, and congenital white spots (macules). It should be noted that mental retardation and seizures may be absent. Facial angiofibromas are almost pathognomonic of TSC; however. inasmuch as these lesions do not appear until late infancy or by 3-4 years of age, this sign is not as valuable in early diagnosis of TSC as are the white macules that are present at birth or early in infancy. Dermatologists usually do not have any difficulty in identifying the type of leukoderma that is present in TSC. However, even when typical white “ashleaf” or “thumbprint” macules are present as the only cutaneous manifestation, it is necessary to confirm the diagnosis. A pediatric neurologist can then evaluate the patient who has white spots with a study of the family members and with imaging and electroencephalography.