Sjogren's syndrome (SS) is an autoimmune pathology which mainly affects salivary and lacrimal glands. Cerebellar degeneration association with SS is very rare, with only a few cases described. The treatment of SS with central nervous system involvement is not consensual. We present a 48-year-old woman with dysarthria, diplopia and ataxia associated with xerostomia. The brain magnetic imaging revealed bilateral cerebellar atrophy. She had antibody positivity for anti-SSA and anti-SSB and minor salivary glands biopsy revealed lymphocytic infiltration. Methylprednisolone, cyclophosphamide and intravenous immunoglobulin yielded no results. Rituximab was initiated with improvement in dysarthria and coordination.