Primary intrasellar schwannomas or neurilemmomas occur rarely and mimic pituitary adenoma, radiologically and clinically. The authors describe the 6th case of primary intrasellar schwannoma mimicking a nonfunctioning pituitary macroadenoma, clinically as well as radiologically. Light microscopically, the present case did not show the typical histology of conventional schwannoma and the confirmative diagnosis was made with the aid of immunohistochemistry and electron microscope. Here, we review the possible hypotheses for pathogenesis of sellar schwannomas on unusual locations.