In the majority of cases, NF1-associated OPGs are classified as WHO grade I pilocytic astrocytomas and only 30–50% of patients show signs or symptoms correlated with the tumor [11,16,17,18,19,20]; in addition, they usually present at a younger age compared to sporadic OPGs in the general population [21] and are characterized by an indolent course, with only one-third of the affected patients requiring a specific treatment [20]. Here, NF1 is linked to neoplasm.