CONTEXT: Tuberous sclerosis complex (TSC) is a genetic disease in the group 
known as neurocutaneous syndromes, with dominant autosomal inheritance. It is 
characterized by skin and adnexal lesions and central and peripheral nervous 
system tumors, with neurological and psychiatric findings. It may affect the 
heart, kidneys, eyes, face, bones, lungs, stomach and dentition.
CASE REPORT: We present the case of a 66-year-old man with dermatological signs 
that included hypopigmented maculae, confetti-like lesions, shagreen plaque, 
angiofibromas on nasolabial folds, neck and back, nail dystrophy and periungual 
fibromas on fingers and toes. An electroencephalogram produced normal results, 
but magnetic resonance imaging showed a nodular image measuring 1.2 x 1.0 cm 
close to the Monro foramen, which was similar to cerebral parenchyma and 
compatible with a subependymal giant-cell astrocytoma. A conservative approach 
was taken, through control imaging examinations on the lesion for seven years, 
with absence of any expansive process or neurological symptoms. Abdominal 
ultrasonography revealed a solid, heterogenic and echogenic mass with a 
calcified focus, measuring 4.6 x 3.4 cm, in the rightkidney, compatible with 
angiomyolipoma. The patient was treated by means of complete nephrectomy because 
of malignant areas seen on histopathological examination and died one month 
after the procedure. This case report illustrates the importance of oral 
clinical findings such as dental enamel pits and angiofibromas in making an 
early diagnosis of TSC, with subsequent screening examinations, treatment and 
genetic counseling.