Klippel-Trenaunay Syndrome (KTS) is a congenital anomaly with multifarious clinical presentations. Present scientific knowledge identifies it to be an unknown mesodermal abnormality acquired during early foetal development. In view of the idiopathic nature of its etiology, it has no preventive or curative options available. This syndrome presents in young age with a triad of clinical signs namely Capillary Vascular Malformations, Soft Tissue and Bone Hypertrophy and Varicose Veins. 

 The clinical management of KTS has been a challenge in phlebology. The present review analyses the etiology, pathology and the efficacy of various treatment options in this  debilitating disease.  There are many exiting recent developments in the management of KTS.  “ foam sclerotherapy  ” and “ gene therapy ”which has the potential for offering high quality of life unlike .