It is important not to misdiagnose Klippel–Trenaunay syndrome with Parkes Weber syndrome (also known as Klippel–Trenaunay–Weber syndrome) that in opposition with the former syndrome, is due to RASA1 mutations and is characterized by the presence of high-flow vascular malformations (191). The gene discussed is RASA1; the disease is capillary malformation-arteriovenous malformation 1.