We describe a patient with soft-tissue and bony hypertrophy of all four limbs. The upper limbs were erythematous, with associated finger deformity. The soft-tissue irregular hypertrophy and engorgement subsided when the limbs were elevated. Retinal examination showed large, dilated, tortuous veins, with no dye leakage on fluorescein angiography. We feel that this case represents an unusual variant of the Klippel-Trenaunay-Weber syndrome.