BACKGROUND: Embryonal tumor with multilayered rosettes (ETMR) is a rare and deadly pediatric central nervous system tumor often seen before the age of 3. ETMR consists of embryonal tumors with abundant neuropil and true rosettes, ependymoblastoma, and medulloepithelioma. The 5-year survival rate has been reported to be between 0% and 30%. Treatment of ETMR is very unstandardized and typically consists of surgical resection, chemotherapy, and radiotherapy. A systematic review was performed to better understand treatment-related outcome trends. METHODS: The authors performed a PRISMA guidelines-based systematic review of the literature. Survival curve analysis using Kaplan-Meier curves and Cox proportional hazards models were used to estimate survival rates between 2 groups and multiple risk factors, respectively. RESULTS: The average survival time was 31.1 months in patients treated with radiotherapy compared to 11.2 months in patients who did not. Radiotherapy was a significant covariate on overall survival ( CONCLUSIONS: Patients treated with radiotherapy in addition to chemotherapy demonstrated a significantly higher survival time. For patients with no metastasis prior to treatment, focal radiotherapy should be strongly considered.