Female of 17 years-old with Klippel-Trenaunay syndrome: port-wine stain, overgrowth of bone and soft tissue in limbs, dental malposition and intellectual disability. Moreover, severe portal hypertension due to portal vein malformation. The Klippel-Trenaunay syndrome includes venous and capillary malformations in the skin, hypertrophy of bones and soft tissue in a limb and, in some patients, lymphatic malformations. Structural abnormalities of the deep venous system can also occur in addition to the key clinical features, although there are few case reports with portal hypertension due to portal vein malformation published.