Neurenteric (NE) cyst is an uncommon developmental lesion lined with epithelium of endodermal origin. To investigate the clinical manifestation and response to surgery, we retrospectively analyzed eight cases of NE cyst that has been confirmed by surgery. Four were in children. The duration of follow-up ranged from 2 to 105 (mean 38) months. One cyst was in the ventral portion of the posterior cranial fossa and the other seven were on the spinal cord. The chief complaints were motor weakness (5), pain (2), and voiding difficulty (1). In one child and three adults, the duration of symptoms was more than 3 years. Children tended to show rapid progression and excellent recovery after surgery. Although total removal of cyst was possible only in two cases, there was no recurrence. The presentation of an NE cyst may be insidious. Clinical suspicion is important for an early diagnosis and better outcome. Because of the benign course after subtotal excision, too-aggressive removal of the lesion should be avoided.