A case report of a patient with Neurofibromatosis 2, linked to the NF2 gene, later developing RMS suggests a possible role of NF2 mutations in RMS development.17 Another patient was diagnosed with hypomelanosis of Ito, a genetic neurocutaneous disorder, and as mentioned previously Neurofibromatosis 1 and Gorlin syndrome have been linked to RMS.22,33 Thus, despite limited genetic testing in the majority of cases, it is reasonable to infer a genetic component could be contributing to PIRMS development. This evidence concerns the gene NF2 and nevoid basal cell carcinoma syndrome.