The PIK3CA c.3140 A>G [p.H1047R] mosaic mutation identified in patient 2 with overgrowth confined only to the right 4th finger diagnosed at birth, has been observed in patients with KTS, CLOVES and very severe FAO, but also in cases of isolated macrodactyly [13]. This evidence concerns the gene PIK3CA and angioosteohypertrophic syndrome.