Klippel-Trenaunay syndrome (KTS) is a rare, sporadic, complex malformation characterized by the clinical triad of (1) capillary malformations (port wine stain); (2) soft tissue and bone hypertrophy or, occasionally, hypotrophy of usually one lower limb; and (3) atypical, mostly lateral varicosity. KTS is a mixed vascular malformation, with predominant capillary, venous and lymphatic components, without significant arteriovenous shunting. Management is largely conservative and the extent of diagnostic evaluation is determined by the planned treatment. Compression is the hallmark of conservative management; laser can be used to treat port wine stains. Imaging before vascular interventions must confirm venous anatomy and deep venous drainage. Techniques for ablation of superficial veins and malformations are individualized and may include sclerotherapy with alcohol or foam, endovenous thermal ablation or, as used most frequently in our practice, surgical stripping and phlebectomy. Intraoperative use of tourniquet will decrease bleeding, selective use of an inferior vena cava filter will prevent pulmonary embolism. A multidisciplinary approach to management of KTS is warranted.