BACKGROUND: Intracranial neurenteric cysts are rare, benign lesions of the central nervous system. OBJECTIVE: To analyze our experience with these lesions and conduct a review of the literature with the aim of identifying a rational approach to diagnosis and management. METHODS: Seven patients underwent surgical treatment for intracranial neurenteric cysts from July 2000 to December 2008. The general strategy was to perform complete resection whenever possible. The clinical, radiological, operative, and pathological findings of the patients were reviewed retrospectively. RESULTS: This series included 3 women and 4 men. The age of hospitalized patients ranged from 19 to 55 years, with an average age of 34 years. Headache and dizziness were the most common chief symptoms. The imaging spectrum for these lesions is broad, leading to several preoperative misdiagnoses. All 7 lesions were resected by a far lateral transcondylar or retrosigmoid approach. Total tumor resection was achieved in 3 patients. Four patients presented transient fever postoperatively. All patients were healthy and showed no signs of tumor recurrence at their latest follow-up. CONCLUSION: Intracranial neurenteric cysts ventral to the brainstem are rare lesions occurring in young adults. These lesions should be considered in the differential diagnosis for intracranial extra-axial cystic lesions anterior to the brainstem. Total surgical resection seems to be the most effective therapeutic method, although radical resection at the risk of impairing the brainstem is not recommended. This benign lesion has a favorable overall prognosis.