This report presents a patient with Devic's neuromyelitis optica associated with primary Sjogren's syndrome. Her first attack was right-sided optic neuritis at age 10 years. Attacks involving both optic nerves and medulla spinalis were recorded during the ensuing years. The diagnosis of Sjogren's syndrome could not be made until the second decade because it was not suspected. (c) 2007 by Elsevier Inc. All rights reserved.