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Klippel-Trénaunay syndrome (KT) associates vessel malformations and overgrowth of a limb. In some cases the vascular malformation may be associated with hypotrophy of soft tissues, that is, of muscles and subcutaneous fat tissue: this syndrome, which has been called inverse KT, could be due to compound heterozygosity with the simultaneous presence of a “plus” and a “minus” allele; post-zygotic recombination could give rise to a homozygous clone for one or the other allele and therefore to hypertrophy or hypotrophy. (2) Alternatively, the KT vascularization disorders could explain either one or the other. In a mild inverse KT there may be no muscle involvement and only congenital localized lipoatrophy (1). The latter can be the only clinical sign and the vessel malformation can only be demonstrated by histological examination of the lipoatrophic lesion (3).