Dry eye syndrome in patients with Sjogren's syndrome is typically associated with severe subjective symptoms, and may lead to corneal complications and visual loss. Autoimmune inflammation of the tear gland and the ocular surface as well as hormonal factors and a neuronal dysregulation are discussed as the main pathogenetic factors involved. Therapy includes safety goggles, humidifiers, correction of lid anomalies, treatment of associated blepharitis, punctum plugs, and the discontinuance of specific medications. Besides numerous artificial tears with various ingredients, and autologous serum, treatment options targeting basic disease mechanisms such as topical steroids and cyclosporin A, oral muscarinic agonists as well as intravenous anti-CD20 monoclonal antibodies (Rituximab) are available. Topical androgens and secretagogues are currently undergoing clinical studies to evaluate their efficacy in the treatment of dry eye.