Chordoid glioma of the third ventricle is an infrequent brain tumour that was described for the first time by Brat et al. in 1998; since then, only 39 cases have been reported. We present a new case of chordoid glioma of the third ventricle in a 51-year-old-man that was treated with total surgical removal, with a good initial postoperative evolution. Sudden death, most likely due to a massive pulmonary embolism, occurred in the third postoperative day. We present the histological characteristics of the tumour and review the literature regarding this entity.