Neurenteric cysts are uncommon, benign lesions that are rarely located intracranially. These cysts are likely due to aberrant embryologic development of the notochord. Clinically, neurenteric cysts may present with symptoms of mass effect, or they can be asymptomatic and incidentally discovered. Imaging features of neurenteric cysts have significant overlap with other intracranial cystic lesions, which can make diagnosis difficult. We present a case of a 35-year-old female with a histopathologically confirmed neurenteric cyst in the premedullary and left cerebellomedullary cistern, with associated symptoms of headache, dizziness, tinnitus, and dysphagia. The patient underwent surgical resection, with improvement in symptoms. We present a review of literature, and a discussion of typical features of multiple intracranial cystic lesions. We hope to promote accurate preoperative diagnosis, to allow for appropriate surgical technique to reduce the risk of recurrence.