Chordoid glioma of the third ventricle is a recently characterized primary neoplasm of the central nervous system. We present a case and discuss the pathologic and radiologic features. We are aware of only 16 other cases documented in the world literature. This radiologic-pathologic correlation alerts pathologists and radiologists to recognize chordoid glioma as a distinct clinicopathologic entity restricted to the third ventricular area of adult patients. (C) 2000 by W.B. Saunders Company.