A risk factor [2] is anything that increases a person’s chance of developing cancer. Although risk factors often influence the development of cancer, most do not directly cause cancer. Some people with several risk factors never develop cancer, while others with no known risk factors do.

The causes of childhood ependymoma are unknown. There is little known about the risk factors or ways to prevent the disease.

Rarely, children with neurofibromatosis type 2 [3] (NF2) have an increased risk of developing ependymoma. NF2 is a hereditary syndrome that causes tumors of the central nervous system that are usually noncancerous.