The case of concomitant occurrence of schwannoma of the ethmoid sinus and meningothelial hyperplasia in female patient without neurofibromatosis-2 is presented. The components of Schwannoma tissue and meningothelial hyperplasia cells islets were confirmed by histopathological and immunohistochemical examinations. Transitional zones of these two different tissues macroscopically as well as microscopically were not observed. Histological and immunohistochemical findings and possible pathogenesis of this uncommon tumor combination is discussed.