Tuberous sclerosis complex (TSC) is a multisystemic disorder that presents an autosomal dominant inheritance pattern and is characterized by the presence of growth of hamartomas (tumors) in the brain, eyes, skin, kidneys, heart and lung.

Objective: To analyze the literature on TSC, its etiology, prevalence, clinical manifestations, diagnosis and treatment.

Methodology: Articles published at MEDLINE/PubMed and ScienceDirect were analyzed, with an emphasis on the last 5 years. Articles were evaluated with the PRISMA and AMSTAR guidelines. The search was carried out using the words "tuberous sclerosis complex", "etiology", “treatment”, “diagnosis” and "clinical manifestations".

Results: TSC is a genetically inherited disorder, with a high but incomplete penetrance, this disorder with multisystem involvement is usually due to mutations in the TSC1 or TSC2 genes. The diagnosis is established with the identification of a mutation of these genes, the treatment for this condition consists of drugs to control the symptoms and sometimes in surgical procedures. The clinical manifestations are heterogeneous, usually manifesting with neurological alterations such as epilepsy, autism, intellectual disability. Dermatological alterations include facial angiofibromas, hypomelanotic macules and nail fibromas. The prevalence is estimated at 1 in 6,000 people, it has been found that it does not have a preference for any sex, and it has a great phenotypic variability, which can make its diagnosis difficult.

Conclusion: TSC is a condition with low incidence, however, dental care is necessary to define therapeutic and preventive management guidelines, which avoid diseases associated with the normal course of the disease and the deterioration of patients.