We report a case of anaplastic ependymoma with extracranial metastases in a 22-year-old female. The patient originally presented with headaches and dysarthria. Neuroimaging revealed a large solid and cystic right fronto-temporal lesion. It was located completely extraventricularly and a glioblastoma was suspected based on the neuroimaging findings. A gross total resection was achieved. Histopathologic examination revealed an anaplastic ependymoma. The patient was treated with radiotherapy. Approximately 1 year after the initial surgery, the patient presented with metastatic disease to the scalp. At 2 years, an intraparotid metastasis was detected. Subsequent neck dissection revealed positive lymph nodes at several levels. It was followed by radiotherapy to the neck. 5 years after the initial surgery, the patient has residual metastatic disease. The case is discussed and the literature on extraventricular ependymal neoplasms is reviewed.