Legius syndrome (LS) is an autosomal dominant condition characterized by cafe au lait spots.

[1] Nearly all individuals with Legius syndrome show multiple café au lait spots on their skin.

[7] Symptoms may include:[2] Features common in neurofibromatosis – like Lisch nodules (iris hamartomas diagnosed on slit lamp exam), bone abnormalities, neurofibromas, optic pathway gliomas and malignant peripheral nerve sheath tumors – are absent in Legius syndrome.

[2] A genetic test is often the only way to make sure a person has Legius syndrome and not neurofibromatosis type I; the similarity of symptoms stem from the fact that the different genes affected in the two syndromes code for proteins that carry out a similar task in the same reaction pathway.

[medical citation needed] Management of Legius syndrome is done via the following:[2][1] The prognosis of this condition is generally considered good with appropriate treatment.