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. Followup 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.