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A case of prenatal Klippel Trenaunay-Weber syndrome is presented, with a description of the sonographically observed disease progression in utero. The appearance of a complex thoracic multicystic mass in association with progressive unilateral lower limb enlargement was strongly suspicious of this disorder. Colour-flow Doppler studies of the cutaneous haemangiomata in utero were not of diagnostic assistance in this case. The prenatal detection of the large cutaneous haemangiomata was of critical importance in minimizing delivery trauma for the mother and fetus.

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