Introduction: Neurenteric cysts are a rare cause of central nervous system compression. Pediatric intracranial cysts are even less common.
Case study: We describe the presentation, imaging features, surgical management, and postoperative course of a 5-year-old female with congenital third nerve paresis, worsening headaches, and a cyst of the third nerve. We performed an eyebrow incision and keyhole supraorbital craniotomy for exploration and subsequent resection of a neurenteric cyst that was densely involved with the third nerve at its exit from the midbrain. Postoperatively, she had chemical meningitis and complete third nerve palsy. This entity is extremely rare in this location but should be included in the differential. We summarize the current literature on these lesions and recommend biopsy and fenestration as surgical treatment.