These findings may, at least in some cases, be a part of the PIK3CA-related overgrowth spectrum (PROS) syndrome [21, 22], encompassing megalencephaly–capillary malformation (MCAP) syndrome, congenital lipomatous overgrowth, vascular malformations, epidermal nevi, scoliosis/skeletal and spinal (CLOVES) syndrome, fibroadipose overgrowth (FAO), and possibly also the Klippel–Trenaunay syndrome (KTS) and Proteus syndrome (PS). Here, PIK3CA is linked to Proteus syndrome.