Objective  To investigate the clinical features and prognosis of intracranial embryonal tumors in children.

  Methods  Clinical data of 27 patients with intracranial embryonal tumors who were admitted to Beijing Shijitan Hospital, Capital Medical University between May 2011 and December 2018 were retrospectively analyzed.

  Results  The study included 27 patients, comprising 17 male and 10 female children, with a median age of 7 years. Twelve patients underwent gross total resection, and 15 patients underwent subtotal resection. After surgery, all 27 patients underwent craniospinal irradiation. The dose of craniospinal irradiation was 27-30.6 Gy, and the dose of the tumor bed was 55.8-60 Gy. Twenty-two patients underwent chemotherapy regimen composed of irinotecan, vtncristine, etoposide, nedaplatin after radiotherapy. The median follow-up period was 22 (4-93) months. Nine patients are alive without local or distant recurrence. Eighteen patients died; the causes of death were intracranial recurrence and spinal cord metastasis. The 1-, 3-, and 5-year survival rates were 79.4%, 50.2%, and 36.5%, respectively.

  Conclusions  Intracranial embryonal tumors in children are rare and malignant and can metastasize along the neuroaxis. Surgery, adjuvant radiotherapy, and chemotherapy are important for patients with intracranial embryonal tumors. Moreover, the prognosis is poor.