Sjögren's Syndrome is a very frequent autoimmune disease, characterised by exocrine gland involvement. Immunologic disorders are also responsive for extraglandular manifestations of the disease, mostly for the digestive involvement. We report a case of primary Sjögren's syndrome with multiple extraglandular manifestations: vasculitis, cryoglobulinaemia, hepatic involvement and presumably neurologic involvement. The particularities of the case are the typical pattern of autoimmune cholangitis, hypogammaglobulinaemia, the absence of antinuclear antibodies and the association with vasculitis. To the best of our knowledge, this is the first case report of autoimmune cholangitis in Sjögren's syndrome