A 56-year-old woman woman with congenital Klippel-Trenaunay-Weber syndrome presented with progressive, fluctuating, right frontal headache and left brachiofacial paresthesias. Clinical examination showed right-sided predominant bone and soft tissue hypertrophy, varicose veins, capillary hemangiomae, and conjunctival varicosities in both eyes, in the absence of neurologic abnormalities (Figure 1).