Widespread primary spinal oligodendrogliomas are a rare variety of tumors that usually affect children. Currently, there are only two adult cases reported worldwide. We report the first case of primary holocord oligodendroglioma with intracranial extension in a young adult female. The patient presented with a 4-month history of fluctuating hemiparesis of the left upper extremity eventually becoming quadriplegic after 1 month. Imaging findings revealed a contrast-enhancing holocord neoplasm spanning from the cervical region to the conus medullaris and with extension to the lower medulla. The patient succumbed to severe pneumonia after 1 month of admission. An autopsy was done and the histopathologic findings were consistent with oligodendroglioma.