Abstract
Chordoid gliomas arise near the third ventricle and commonly present around 40 years of age. These rare tumors are non-invasive and often present with headaches and visual disturbances. Contrast enhancement on MRI is typical for these tumors and immunohistochemical (IHC) staining is positive for glial fibrillary acidic protein (GFAP). Surgical resection is the treatment of choice. We present this case of chordoid glioma because of its unique characteristics. The tumor lacked contrast enhancement on MRI and demonstrated juxtanuclear dot-like immunoreactivity for synaptophysin which is a feature not previously reported in the literature. It is important for pathologists and radiologists to be on the lookout for atypical presentations of these rare tumors.