Chorioretinal folds are a rare presentation of a meningioma. We report a case of a meningioma with chorioretinal folds as its initial presentation. A 39-year-old female patient complained of progressive blurring of the right eye. A fundus examination of the right eye revealed chorioretinal folds. Magnetic resonance imaging found a tumor mass involving the right orbit and right anterior middle cranial fossa. An echo-guided biopsy confirmed the meningioma. She received surgical excision of the meningioma, and her visual acuity was stationary at 12/20 after 15 months of follow-up. We review the etiologies of chorioretinal folds and elucidate the strategies for dealing with them. Certain underling diseases associated with chorioretinal folds are lethal and require prompt management. Ophthalmologists should evaluate these patients carefully in a stepwise manner.