[A woman with a vulvar swelling, a rare manifestation of the Klippel-Trenaunay-Weber syndrome].

A 32-year-old woman presented with a vulvar swelling. The swelling had always been there but had increased after her last pregnancy, 5 years earlier. Because of progressive complaints, such as pain and a feeling of pressure, she now wished surgical correction. The swelling turned out to be varicosis of the right labium majus associated with the Klippel-Trenaunay-Weber syndrome. The vulvar swelling was removed by electrocoagulation. The pathological diagnosis was 'venous haemangioma'. The Klippel-Trenaunay-Weber syndrome is a congenital skin condition in which vascular angiomas, varicosis and trophic changes in the soft tissue and skeleton can arise. Vulvar involvement is rare. The abnormality can be removed surgically, for example by electrocoagulation. There is a chance of recurrence.