The association of varicose veins, soft tissue and bony hypertrophy and cutaneous hemangioma of the "port-wine" variety confined to one extremity was first reported in 1900 by Klippel and Trenaunay. However, Parkes-Weber also described a second entity which appears to be similar to the Klippel-Trenaunay triad with addition of an arteriovenous fistula. It is proposed that the syndrome originally described by Klippel and Trenaunay in 1900 without arteriovenous fistula be considered as a specific entity, the Klippel-Trenaunay syndrome. We have experienced rare varient form of this syndrome, especially in the aspect of orthopaedics.