The ISSAVA classification for vascular anomalies associated with other related PIK3CA mutation disorders includes, aside from KTS, several others syndromes, such as macrocephaly; the capillary vascular malformations of the lower lips; the capillary lymphatic malformations of the head and neck; the disproportions of the face and limbs, partial or generalized overgrowth involving one or more body segments (CLAPO) syndrome; the congenital lipomatous, overgrowth, vascular malformations; and epidermal nevi (CLOVES) syndrome [15,16,17]. Here, PIK3CA is linked to CLAPO syndrome.