Klippel-Trenaunay-Weber syndrome with hemimegalencephaly

There are many documented cases of Klippel-Trenaunay-Weber syndrome characterized by the triad of port wine stain, varicose veins, and hypertrophy of bones and overlying tissue. A few cases have even included the additional findings of hemimegalencephaly. We report a case of Klippel-Trenaunay-Weber syndrome with hemimegalencephaly and calvarial hypertrophy, but no evidence of limb or trunk hypertrophy.