Abstract Background Pediatric low-grade gliomas (pLGG) are the most common childhood solid tumors. While typically indolent with excellent prognoses, certain mutations have been associated with aggressive tumor behavior. Case Presentation A 19-month-old with biopsy-confirmed pLGG experienced symptoms and progression of residual tumor two months following debulking surgery. Repeat debulking and biopsies similarly confirmed pLGG, while molecular analyses identified a FGFR1:TACC1 fusion. Discussion While rare, pLGG can exhibit aggressive tumor biology. This review highlights the role of FGFR aberrations in pLGG characterization, prognostication, and clinical decision-making. An examination of targeted therapies and ongoing clinical trials for recalcitrant pLGG was also performed.