ObjectiveTo review the clinical records of patients with central nervous system (CNS) embryonal tumors, not otherwise specified (NOS); and summarize their clinical features, diagnosis, and treatment.MethodWe reviewed the data of patients with intracranial tumors admitted to Department of Neurosurgery of West China Hospital, Sichuan University from January 2014 to December 2016, and retrospectively analyzed the clinical features, diagnosis, and treatment of seven patients with CNS embryonal tumors, NOS.ResultsThere were 4 males and 3 females, and the mean age was 25.4 years old. The tumor was located in cerebral hemisphere in 5 patients, and in third ventricle in 2. Clinical presentation included headache, nausea, and vomiting due to intracranial hypertension, and focal neurological signs. All patients underwent craniotomy for tumor resection and postoperative pathology confirmed CNS embryonal tumor, NOS. The patients were followed up for 6 months to 3 years, and 2 patients died during follow-up.ConclusionsCNS embryonal tumor, NOS is malignant intracranial lesion, and has been enlisted as a separate entity under classification of CNS embryonal tumors. It has its unique radiological features, including rare occurrence of perilesional edema, cystic changes, and clear demarcation. Through comprehensive treatment including surgical resection, chemotherapy, and radiation therapy, patients can enjoy prolonged survival and improved quality of life.