Because NF-1 can be expressed clinically in various manners, differing even between members of the same family carrying the same NF1 mutation, treatment may range from clinical observation and isolated removal of cutaneous tumors to drug-centered therapy for neurological and endocrinological abnormalities and chemotherapy or targeted therapy for conditions such as optic gliomas, malignant peripheral nerve sheath tumors, and GIST [8,9,23,24,25,26,27,28,29]. Here, NF1 is linked to malignant peripheral nerve sheath tumor.