The chordoid glioma is a recently recognized rare neoplasm of the third ventricle and hypothalamus and, as the name implies, has a chordoid appearance. Using histological, immunohistochemical and ultrastructural studies, we report a case of a 32-year-old man with a chordoid glioma in the third ventricle. Magnetic resonance T1-weighted images of the brain with homogenous contrast enhancement revealed a well circumscribed, isointense mass occupying the third ventricle. Histologically, the tumor showed cords and clusters of epithelioid cells within a mucinous background along with lymphoplasmacytic infiltrate, and appeared to be reminiscent of a chordoma or chordoid meningioma. Immunohistochemically, the tumor cells were immunoreactive for glial fibrillary acidic protein, vimentin and CD34 but negative for epithelial membrane antigen. Ultrastructural study revealed round to spindle shaped cells with abundant cytoplasmic intermediate filaments and cytoplasmic zonation.