Pilomyxoid astrocytoma (PMA) is a recently defined pediatric brain tumor; PMAs were previously classified within the pilocytic astrocytoma (PA) category. Nevertheless, PMA has different histological features and has been shown to behave more aggressively than PA. These findings indicate that PMA may be a unique entity that is distinct from PA, or it may be an unusual variant. To increase awareness of PMA within the neurosurgical community, the authors review the diagnostic criteria, prognostic implications, and current management of this recently described pediatric low-grade astrocytoma.