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Background: Meningiomas correspond to only 8% of pineal region tumors. Chordoid meningioma (CM) comprises only 0.5 to 1.0% of all intracranial meningiomas and is even rarer in the pineal region. In the literature, we found only six cases of pineal region CMs reported. Clinical presentation: We describe a case of one patient with headache, confusion, slurred speech and gait disturbance. The ophthalmologic examination revealed vertical gaze paresis and near-light dissociation of papillary reflex. MRI disclosed a large pineal region tumor with heterogeneous contrast enhancement. The tumor was totally removed by an occipital transtentorial approach (OTA) in a “three-quarter prone” position. Histological analysis disclosed a CM. Conclusion: Chordoid meningioma is a very rare variant of meningioma and the pineal region is a poorly described site. This study contributes to the understanding of this heterogeneous entity, to consider the CM as a differential diagnosis of pineal region tumors and to provide proper management of affected patients.