AIM: Intraventricular glioblastomas (IVGBMs) are rare tumors within the central nervous system characterized by unique challenges in diagnosis and management due to their location within the ventricular system. Despite their rarity, these tumors necessitate comprehensive study to refine diagnostic approaches and optimize therapeutic strategies. METHODS: A systematic review was conducted using PubMed, Scopus, Web of Science, and Google Scholar databases to identify relevant literature published up to January 2024. Inclusion criteria encompassed studies in English focusing on clinical characteristics, radiological features, pathology, and treatment of IVGBM. Data synthesis and analysis followed Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines. RESULTS: Twenty-four articles met the inclusion criteria, comprising 47 patients with IVGBM. The median age was 47 years, with a male predominance (32 males, 15 females). Common symptoms included increased intracranial pressure and seizures. Tumors predominantly affected the lateral ventricles (body and trigone). Surgical resection (subtotal or gross total) was the primary treatment approach, with adjuvant therapies (radiotherapy, chemotherapy) administered postoperatively. CONCLUSIONS: IVGBM present distinct diagnostic and therapeutic challenges due to their ventricular location. Current treatments primarily involve surgical resection followed by adjuvant therapies, though outcomes remain guarded. Further research is needed to enhance understanding and management of this rare glioblastoma subset.