Chemosis of the eyes is usually attributed to carotid cavernous sinus dural arteriovenous fistulae. Herein, we reviewed unusual cases in which chemosis of the eyes originated from dural ateriovenous fistulae (dAVFs) that were distinctly different from carotid cavernous sinus fistulae. Cases in which ocular symptoms were related to increased intracranial pressure either due to sinus thrombosis or cortical venous drainage without involvement of superior or inferior ophthalmic veins were excluded in this review. Several different types of dural AVFs were associated with chemosis, and these included dAVFs harboring a feeding artery from branches of the external carotid artery directly draining to the superior ophthalmic vein or cavernous sinus via the superior petrous sinus, posterior fossa dAVFs draining via the inferior petrous sinus and cavernous sinus to the ophthalmic vein, a fistula between the ophthalmic artery or branches of the internal carotid artery and inferior ophthalmic vein, or tentorial fistula with a drainage vein to the cavernous sinus via the vein of Galen. This study reviews the symptomatology, treatment options, and cerebrovascular abnormalities observed for these unusual dAVF's with chemosis.