OBJECTIVE: To retrospectively analyze the clinical data of 6 patients with foramen magnum (FM) neurenteric (NE) cysts, and summarize the clinical characteristics and treatment experience for this rare disease in our single center. METHODS: Between January 2011 and December 2015, 6 patients with FM NE cyst were surgically treated at Xuan Wu Hospital of Capital Medical University. We summarize the treatment experience of these patients through a retrospective review of the clinical information, imaging features, surgical details, and follow-up outcomes. RESULTS: All 6 patients were female, ranging in age from 15 to 54 years (mean age, 36.8 +/- 12.9 years). Occipital headache along with cranial nerve injury were the most common symptoms. Preoperative brain magnetic resonance imaging identified all lesions in the FM region, with an oblong or lobulated shape. The surgical approach was far lateral in 4 patients and suboccipital midline in 2 patients. Total lesion removal was completed in 4 patients, and subtotal excision was performed in the other 2 patients, in whom the cyst wall was intensely adherent to surrounding structures. In all 6 patients, the preoperative symptoms were significantly relieved after surgery. No recurrence was seen after a mean follow-up of 27.3 months (range, 3-70 months). CONCLUSIONS: Our present study identified a female predominance among patients with intracranial FM NE cyst. Surgical excision is the optimum treatment strategy for this rare disease. Our findings indicate that subtotal removal of an FM NE cyst may be associated with favorable outcomes, but strict long-term follow up is needed.