The intramedullary anaplastic ependymoma rarely occurs in the cervicomedullary junction. A 45-year-old woman had a history of right arm pain for several months. Magnetic resonance imaging (MRI) of the cervical spine demonstrated an intramedullary tumor with syrinx at the cervicomedullary junction. The patient underwent a partial resection at another institute. Neurologic deficit worsened after the first surgery. The follow up MRI showed that the enlarged enhancing tumor and syrinx still existed with the same size and configuration. Complete surgical resection was achieved in the revision surgery. Final histologic examination confirmed the diagnosis of an anaplastic ependymoma, and since complete surgical resection was achieved the patient did not receive adjuvant radiation or chemotherapy. The patient was followed-up periodically at the outpatient department, and at the 7 months follow-up the muscle tone of the right hand was normal but with mild sensory deficit, and the MRI demonstrated no evidence of recurrent disease. Intramedullary anaplastic ependymoma that occur in the cervicomedullary junction which are completely resected may be followed-up without adjuvant radiation or chemotherapy to attain good clinical outcome.