Atypical teratoid/rhabdoid tumors (AT/RTs) are rare, highly aggressive World Health Organization grade 4 brain tumors associated with early cerebrospinal fluid dissemination (CSF). AT/RTs typically occur in children under 4 years of age however, can also occur in adults and often carry a poor prognosis. These tumors are exceptionally uncommon, with evidence limited to isolated case reports. We illustrate a case of hemorrhagic left lateral ventricular AT/RT in a 5-year-old boy and systematically synthesize global literature evaluating clinical presentations, therapeutic interventions, and survival outcomes of primary supratentorial intraventricular AT/RT. Systematic searches were executed across PubMed, Embase, and Scopus databases up to 31 May 2026. Studies tracking histologically or immunohistochemically confirmed pediatric intraventricular AT/RT were included. Study quality was evaluated qualitatively via Joanna Briggs Institute tools; qualitative descriptive analysis synthesized outcomes, supplemented by post hoc stratified subgroup analysis. Twenty-four descriptive, uncontrolled studies comprising 28 pediatric patients (mean age: 3.9 years) were synthesized. The lateral ventricle was the primary epicenter (53.5%). Surgical resection margins were divided between gross total resection (GTR; 64.2%), subtotal resection (STR 17.8%), and biopsy (7.1%). The cohort demonstrated a 43.0% disease progression rate and a 69.6% (16/23) overall mortality rate among patients with follow up. Descriptive subgroup analysis demonstrated a comparable high mortality across age groups and tumor locations, whereas mortality was highest among patients with metastatic disease and appeared lower after GTR than STR 33.3% (6/18) vs. 80.0% (4/5).