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Introduction and importance: Chordoid glioma is a rare, slow-growing glial tumor that most commonly arises from the anterior third ventricle or suprasellar region. Due to its rarity, deep location, and non-specific clinical and radiological features, preoperative diagnosis and surgical resection remain challenging. Case presentation: We report the case of a 57-year-old woman who presented with a 1-month history of progressive headache and blurred vision. Magnetic resonance imaging demonstrated a well-circumscribed, homogeneously enhancing mass in the suprasellar region with upward extension to the floor of the third ventricle, causing obstructive hydrocephalus. The patient underwent microsurgical transcranial tumor resection, and gross total resection (GTR) was achieved. Histopathological examination and immunohistochemical analysis confirmed the diagnosis of chordoid glioma, WHO grade II. Postoperatively, the patient showed marked clinical improvement. During follow-up, visual symptoms improved, and no new neurological deficits were observed. At 6 months, imaging revealed no evidence of tumor recurrence. Clinical discussion: Due to its deep-seated location within the third ventricle and firm consistency, GTR of chordoid glioma remains challenging. We report the first successful GTR of this tumor in Vietnam. The surgical approach, clinical and radiological features, and postoperative follow-up are discussed with a review of the literature. Conclusions: This report presents the first successful surgical resection of a chordoid glioma in Vietnam. Although rare, chordoid glioma should be considered in the differential diagnosis of suprasellar and third ventricular tumors. Surgical resection remains the cornerstone of treatment, and favorable outcomes can be achieved with appropriate surgical planning and follow-up.