RESUMO
The term “Neurocutaneous syndromes” also known as Phakomatoses refers to a broadcategory of congenital diseases that includes anomalies of neuroectodermal and occasionallymesodermal development, hence commonly involving the skin, eyes and central nervoussystem. The term Phacomatoses was coined by Van Der Hoeve in 1921. Tuberous sclerosisis one type of Neurocutaneous syndromes. The word "tuberous sclerosis" comes from thedistinctive cortical tubers that Désiré-Magloire first described. Tuberous sclerosis is ahereditary disorder that affects numerous body systems. A 30 year old female diagnosed asTuberous sclerosis was referred by Dermatology Department. Detailed ophthalmicexamination was done. Patient was diagnosed with Adenoma sebaceum of upper eyelids inboth eyes and multiple retinal hamartoma in left eye.