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Klippel-Trenaunay syndrome (KTS) is a rare congenital disease, characterized by a triad of clinical features: (1) capillary malformations, manifesting as a "port wine stain", (2) vascular anomalies, mostly varicose veins and (3) bone and/or soft tissue hypertrophy, usually of one lower extremity. The symptoms are frequently accompanied by lymphatic abnormalities that in some cases may lead to lymphedema. KTS is mostly benign in the course. Nevertheless, patients with KTS are at higher risk of developing deep vein thrombosis (DVT), pulmonary thromboembolism, recurrent episodes of thrombophlebitis, dermatolymphangitis or internal bleeding. Management in KTS should be individualized, minimally invasive and involve multidisciplinary care of the patient. We report a case of a man with fully symptomatic KTS, after incident pulmonary thromboembolism in the past and with severe phlebolymphedema, effectively treated with decongestive lymphatic therapy (DLT). We also provide a review of the literature on the clinical aspects of this complex syndrome.

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