<p>Klippel-Trenaunay syndrome (KTS) is a rare congenital disorder of blood and lymphatic vessels that is characterised by a combined vascular malformation of the capillaries, veins and lymphatics, congenital abnormalities, and associated limb hypertrophy. This review provides an overview of the disease with emphasis on the management and underlying aetiology of the syndrome. Management of this condition is largely conservative and requires a multidisciplinary approach. In the majority of cases a conservative approach is indicated with compression as the mainstay of conservative management. Laser can be used to treat port wine stains. Serious complications can occur and regular surveillance is necessary.</p>
