A female patient with Klippel-Trenaunay syndrome, including hypertrophic bone and soft tissue in the forelimbs, bilateral lower limbs lymphedema, port-wine stains, and superficial veins of Servelle, was presented. The diagnosis of lymphedema was confirmed by lymphoscintigraphy and indocyanine green lymphography. The novel treatments consisted of vascularized lymph node transplantation to the left lymphedematous extremity and lymphovenous anastomosis to the right lymphedematous extremity. Significant improvements in subjective and objective clinical outcome were observed early in the postoperative period with continued improvements during the follow-up period.