Purpose: Neurenteric cysts are rare congenital remnants formed by a failure of separation between endoderm and ectoderm in utero.
Methods: We describe a case of a 7-month-old male with a large cervical neurenteric cyst presenting with intermittent neck stiffness and irritability.
Results: This cyst was resected, recurred, and required repeat surgery. The patient's postoperative course included aseptic meningitis and hydrocephalus requiring ventriculoperitoneal shunt and later management of tethered cord, necessitating detethering.
Conclusion: Unique features of this case include the presence of intermittent pain symptoms, which may be attributable to cyst filling and emptying. Hydrocephalus is an uncommon finding that may be secondary to aseptic meningitis from cyst rupture. Tethered cord is also an unusual entity that can accompany this diagnosis, warranting additional imaging work-up and monitoring.
Keywords: Aseptic meningitis; Congenital; Enterogenous cyst; Hydrocephalus; Neurenteric cyst; Tethered cord.