[Localized xanthoma in lymphedema]

We report a case of xanthoma and lymphoedema in a patient who has aortic insufficiency. The association of lymphoedema and vascular malformations has been reported and seems to be occasional. The lymphoedema is primary and of early onset. Localized xanthomas follow the ingestion of lipoproteins by local histiocytes. We observed partial regression of xanthomas with alleviation of lymphoedema. There seems to be the ten reports of such association.