Association of bone and soft tissues hypertrophy, cutaneous haemangioma and superficial varicosity classically form the Klippel-Trenaunary-Weber syndrome. One case was diagnosed in a 12-year-old patient, admitted for a right lower limb congenital oedema. The goal of this study is to attract clinicians' attention upon this rare affliction with a difficult diagnosis and treatment. Clincally generalised oedema of the right limb was associated with voluminous superficial varicsity. A thrill was present at the internal side of the thigh, with a systolic murmur, indicating an arteriovenous fistula. Phlebography showed dilatation of deep venous network, compressin of the right superficial fermoral vein and doubling left deep femoral vein. Muscular echography revealed the presence of a compressing mass of tissues at the thigh level, which motivated the surgical procedure. It was a muscle in abnormal position trappng the deep vein, which was freed after muscle resection. The patient died 4 days post operatively, from massive pulmonary embolism. Diagnosis of Klippel-Trenaury-Weber syndrome must be considered when facing any congenital lower limb hypertrophy. Phlebography showing deep vascular lesions is an essential method of diagnosis. When surgical treatment is considered, the prevention of thromboembolic complications has to be instituted.
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