As the variant p.(Asn549Lys) in FGFR2 has previously been linked to an endometrial endometrioid adenocarcinoma (Dutt et al. 2008), we hypothesize that the presented mosaic FGFR2-associated neurocutaneous syndrome is associated with an increased tumor risk, although further cases are needed to verify this. This evidence concerns the gene FGFR2 and endometrial endometrioid adenocarcinoma.