INTRODUCTION: Spinal arachnoid cysts are relatively uncommon; it is most frequently seen on thoracic spine level and posterior in location. Anterior cervical intradural arachnoid cysts are even rarer, and only a few cases were reported internationally. The cysts are usually asymptomatic but may present with progressive cord compression.

CASE: We report on a case of a 4-year old male presented with a 3-month history of neck pain, limitation of movement and progressive quadriparesis. There are various etiologic diagnosis to consider in cervical cord compression, however, for this patient, the MRI result suggests of an arachnoid cyst at the upper cervical level. Surgery is necessary and C2 Hemilaminectomy and complete excision of anteriorly located arachnoid cyst were done. The neurologic course is dramatic but early surgery provided a favorable outcome and excellent return of neurologic function.