Genetic causes for gangliogliomas are not yet known. There are rare reports of gangliogliomas co-occurring with neurofibromatosis type 1, neurofibromatosis type 2, Down syndrome, Turcot syndrome, Fragile X syndrome and Peutz-Jegher syndrome. It is unknown if these rare reports of co-occurrence are by chance or suggest a genetic cause for gangliogliomas.
A family history is not expected due to sporadic occurrence, genetic abnormalities if present, are likely acquired somatic genetic abnormalities, without risk of transmission to offspring.