Objective The purpose of this study was to retrospectively analyze the main factors affecting the prognosis of pediatric intracranial embryonal tumor with multilayered rosettes (ETMR) and to provide effective suggestions for improving the prognosis of children with this condition. Methods From January 2013 to December 2023, clinical data were collected from 29 patients with ETMR who underwent surgery and were diagnosed postoperatively. Data were obtained from medical records and telephone follow-ups. A total of 17 patients who completed follow-up were included in the analysis. The clinical data of these 17 children with ETMR were retrospectively reviewed, summarizing their gender, age, tumor location, clinical manifestations, extent of surgical resection, postoperative radiotherapy and chemotherapy, recurrence and metastasis, as well as prognosis. Results Among the 17 patients, 11 were diagnosed with ETMR, 2 with embryonal tumor with abundant neuropil and true rosettes (ETANTR), and 4 with medulloepithelioma (MEPL). The median age at diagnosis was 2.5 years (range: 11 months and 20 days to 8.6 years). The study endpoint was death, with a median survival time of 8 months (range: 0 to 130.5 months). The 1-year progression-free survival (PFS) and overall survival (OS) rates were 18% and 41%, respectively, while the 3-year PFS and OS rates were 18% and 24%, respectively. Thirteen patients (76%) died due to tumor-related complications, progression, or recurrence. Survival analysis showed that gender, postoperative radiotherapy, chemotherapy, and combined chemoradiotherapy had statistically significant effects on overall survival (P<0.05). Conclusion ETMR is a highly invasive tumor with a high mortality rate. Treatment typically begins with maximum surgical resection. Gross total resection, postoperative radiotherapy, chemotherapy, or combined chemoradiotherapy are associated with better prognosis.