Sjögren syndrome (SS) is an autoimmune disease, rarely affecting the central nervous system. The case is reported on a 68-year-old male patient who presented with keratoconjunctivitis sicca and xerostomia, during neuropsychological evaluation showed moderate to severe multidomain cognitive impairment. The magnetic resonance showed evidence of hypertrophic pachymeningitis. The CSF study showed pleocytosis, mild proteinuria, and negative cultures. The immune positive profile, Anti SS-A, SS-B and salivary gland biopsy was compatible with SS. The treatment was started with methylprednisolone pulses at a dose of 1 g every 24 h for three days, and controlled with maintenance therapy, showing a favorable response. Chronic meningitis is a diagnostic challenge; among the less common causes is hypertrophic pachymeningitis in SS, and less frequently in male patients (1/9). Non-specific neurological manifestations should be considered, such as headaches or neuropsychiatric symptoms, as on some occasions rare cases may be encountered, such as the one described.