INTRODUCTION: The etiological diagnosis of recurrent aseptic meningitis often requires difficult investigations. We report a case of recurrent aseptic meningitis associated with a primary biliary cirrhosis. CASE REPORT: A 37-year-old woman presented four recurrences of isolated meningitic syndrome with fever. Intravenous steroid treatment was effective. Investigations revealed an old right tympano-labyrinthine fracture with, on MRI, an abnormal enhancement of the posterior wall of the internal auditory canal. Serum tests were also significantly positive for antimitochondria antibodies subtype M2. CONCLUSION: Definitive diagnosis was recurrent puriform aseptic meningitis secondary to an inflammatory non-septic ears-nose-throat focus in a latent autoimmune context of primary biliary cirrhosis. To our knowledge, this is the first case reported in literature.