Molecular analysis of FGFR2 should especially be considered in the genetic workup of individuals with the suspected diagnosis of a mosaic neurocutaneous condition such as BSTV syndrome, ECCL, or SFM syndrome, as the knowledge of the molecular cause might have relevant implications for genetic counseling, prognosis, tumor surveillance, and potential treatment options. This evidence concerns the gene FGFR2 and encephalocraniocutaneous lipomatosis.