PURPOSE: To describe a case of an occipital arteriovenous malformation (AVM) presenting with unilateral signs mimicking a carotid-cavernous sinus fistula (CCF). DESIGN: Interventional case report. METHODS: A 67-year-old normotensive male presented with loss of vision, pain, and proptosis of the right eye. Best-corrected visual acuity was right eye 6/36 and left eye 6/5. Examination of the right eye revealed orbital congestion with arteriolization of the episcleral vessels and an intra-ocular pressure of 44 mmHg which was refractory to medical treatment. RESULTS: Computed tomography (CT) angiogram and cerebral angiography found an occipital AVM draining into the right sphenoparietal sinus and thereafter the right ophthalmic vein, by passing the cavernous sinus. Embolization followed by excision of the AVM resulted in recovery of vision, reversal of proptosis, and normalization of intraocular pressure. CONCLUSIONS: Rapid diagnosis and immediate intervention resulted in a rewarding visual recovery despite a persisting left homonymous hemianopia. PURPOSE: To describe a case of an occipital arteriovenous malformation (AVM) presenting with unilateral signs mimicking a carotid-cavernous sinus fistula (CCF). METHODS: A 67-year-old normotensive male presented with loss of vision Best of corrected visual acuity was right eye 6/36 and left eye 6/5. Examination of the right eye revealed orbital congestion with arteriolization of the episcleral vessels and an intra-ocular pressure of 44 mmHg which was refractory to medical treatment. RESULTS: Computed tomography (CT) angiogram and cerebral angiography found an occipital AVM draining into the right sphenoparietal sinus and thereafter the right ophthalmic vein, by passing the cavernous sinus. Embolization followed by excision of the AVM draft in recovery of vision, reversal of proptosis, and normalization of intraocular pressure. CONCLUSIONS: Rapid diagnosis and immediate intervention resulted in a rewarding visual recovery despite a persisting left homonymous hemianopia