Usual locations of arachnoid cyst are the middle cranial fossa in 50-60%, cerebellopontine angle (10%) and suprasellar area (10%). Most of these malformations are asymptomatic. Premedullar arachnoid cysts are extremely rare. All previous cases reported were operated. We report a case of an asymptomatic giant craniocervical junction arachnoid cyst with a follow up of five years. In 2002, an adolescent consulted for persistent cervical pain. Encephalic MR showed a giant ventral craniocervical junction arachnoid cyst. Neurologic examination was normal. Conservative treatment was decided with a clinical follow up and repeated MR in case of persistent cervicalgia. Craniocervical junction arachnoid cysts are anecdotic. Medical care cannot be standardized. Pathogenesis and management are discussed.