Rosai-Dorfman disease (RDD) is an uncommon pathologic condition of unknown ethiotogy with an idiopathic proliferation of the hystiocytes. It is generally presented with massive bilateral hypertrophy of the cervical lymph nodes. But other lymph nodes may also be involved. Approximately, 30% of these patients have extra nodal mass or lesion with different signs or symptoms depending on localization. We present two mate siblings with Rosai-Dorfman disease who have classical cervical lymphadenopathy associated with progressive neurosensorial hearing toss and dural-based intracranial lesions. (C) 2004 Elsevier Ireland Ltd. All rights reserved.