To evaluate the long-term outcome and functional recovery of intramedullary medullocervical ependymoma (IME), the clinical charts of 38 surgically treated consecutive cases of IME were reviewed. Follow-up was obtained prospectively. The mean age of the patients (19 male and 19 female) was 35.3 years (range: 11-60 years). Complete resection was achieved in 33 (86.8%) patients. Fourteen patients worsened postoperatively; five and seven of these improved to their baseline levels within 1 and 3 months, respectively. By 1 year postoperatively, 17 patients returned to work. After a mean follow-up duration of 81.5 months, 31 patients improved or stabilized, and 3 had recurrence. The means of the modified McCormick grade (mMG) scores before the operation, at discharge, 1 year after the operation, and at the most recent evaluation were 1.76, 2.13, 1.82, and 1.84, respectively. A favorable long-term outcome of the mMG was associated with a good preoperative status (mMG I) (odds ratio [OR] = 9.956, p = 0.008) and well-defined tumor boundary (OR = 7.829, p = 0.035). Improvements in the postoperative walking dysfunction and paresthesia over time were associated with the absence of preoperative walking dysfunction (p = 0.047) and paresthesia (p = 0.028), respectively. The 12-year progression/recurrence-free survival and overall survival rates were 92.0% and 93.7%, respectively. The study suggests that the goal of surgery is to stabilize the preoperative neurological function and that a favorable outcome may be achieved in patients with good preoperative statuses and well-defined tumor boundaries. Surgery should be performed as soon as possible after the diagnoses and before the neurological functions deteriorate.