This review will explore major neurocutaneous syndromes frequently encountered in clinical practice, including neurofibromatosis types I and II (NF1 and NF2), von Hippel-Lindau (VHL) syndrome, tuberous sclerosis complex (TSC), hypomelanosis of Ito (HI), Sturge-Weber Syndrome (SWS), hereditary hemorrhagic telangiectasia (HHT), ataxia-telangiectasia, and cerebrotendinous xanthomatosis. Here, NF2 is linked to Sturge-Weber syndrome.