Tentorial dural arteriovenous fistula(dAVF)is classified as Cognard 4 with a high risk of aggressive feature, such as intracranial hemorrhage, venous infarction, and intracranial hypertension. We report a rare case presenting with ocular symptoms caused by tentorial dAVF. A 59-year-old man presented with progressive chemosis and exophthalmos of his left eye. Angiography showed a tentorial dAVF that was primarily fed by the middle meningeal artery and the meningohypophyseal artery. The AVF drained into the superior ophthalmic vein and the cerebellar cortical vein via an enlarged petrosal vein. Because transarterial Onyx embolization resulted in incomplete obliteration of the fistula, he underwent microsurgery via a suboccipital approach to obliterate the shunt. Postoperative angiography showed complete obliteration of the shunt. His ocular symptoms rapidly cured. We present this rare case and discuss the pathomechanism associated with the development of ocular symptoms secondary to a tentorial dAVF from the viewpoint of microvascular anatomy.