Tuberous sclerosis (TS) is a genetic disorder affecting multiple body systems, 
and resulting from alterations in cell differentiation and proliferation. The 
disease is characterized by the development of benign hamartomatous tumors: 
neurofibromas and angiofibromas, located in the skin, central nervous system, 
mucosas and other organs. Abnormal neural cell migration plays an important role 
in the neurological dysfunctions found in TS, the predominant features being 
mental retardation, seizures and behavioral disorders. The condition is produced 
by mutations in genes TSC1 of chromosome 9q34 and TSC2 of chromosome 16p13.3, 
and exhibits a dominant autosomal hereditary trait--though 60-70% of cases are 
sporadic and represent new mutations. The phenotype is highly variable. The 
prevalence of TS varies between 1/6000 and 1/10,000 live births. The present 
study reports the case of a 21-year-old male with TS and oral manifestations of 
the disease. The clinical characteristics are described, along with the 
diagnostic criteria and the management strategies, with a review of the 
literature on the disease.