Background:Atlantoaxial cysts are rare, and only 46 histologically confirmed cases have been reported.Case Description:A 75-year-old male presented 2 years ago with headache, neck pain, loss of balance, and episodic dysphagia, for which he had undergone posterior cervical drainage of a left-sided atlantoaxial cyst. Although his original symptoms resolved, they recurred 2 years later and were correlated with an enhanced MR that showed a recurrent left C1-C2 synovial cyst causing marked cervical cord compression. It was successfully resected through a navigation-guided, endoscope-assisted posterior approach. The patient's symptoms/signs resolved completely, and he has remained symptom-free for over 30 months postoperatively, with no evidence of recurrence on MR or craniocervical instability.Conclusions:A patient who successfully underwent resection of a recurrent synovial cervical cyst using a navigation-guided, endoscope-assisted posterior approach has been reported here.