The authors describe the case of an 11-year-old boy with no history of prior trauma, who experienced acute proptosis and visual loss while showering. Diagnostic carotid artery angiography revealed an orbital lymphatic-venous malformation (LVM) concomitant with an intraorbital arteriovenous fistula. The patient was treated with transvenous coil and N-butyl cyanoacrylate glue embolization, combined with direct percutaneous glue injection into the LVM followed by excision. There was good clinical and radiological response without recurrence at the 2-year follow-up. Diagnostic carotid angiography should be considered in cases of orbital LVM, as a concomitant arteriovenous fistula might be present in rare cases.