Key Points
•	IVGBMs are a rare and aggressive subset of glioblastomas, arising primarily from the neuroglial cells of the septum pellucidum or fornix and extending into the ventricular system.
•	Diagnosing IVGBMs requires advanced imaging techniques like MRI, alongside histopathological confirmation, due to their similarity to other intraventricular tumors.
•	Surgical resection, preferably GTR, is the cornerstone of treatment, but complete removal is challenging due to the tumors' infiltrative nature and proximity to critical brain structures.
•	Prognosis for IVGBM patients is generally poor, with survival rates like parenchymal GBMs.
•	Molecular and genetic profiling of IVGBMs reveals common alterations in genes such as TP53, EGFR, and PTEN.
•	Ongoing research and advancements in imaging, surgical techniques, and adjuvant therapies are essential for improving diagnostic accuracy, treatment efficacy, and survival outcomes for patients with IVGBMs.