Orbital arteriovenous fistula is a rare acquired disorder. The coincidence of arteriovenous fistula with lymphaticovenous malformation is even rarer. The optimal treatment, thus, is controversial. Surgical approaches vary widely, with associated pros and cons. The purpose of this case report is to describe an orbital arteriovenous fistula in a 25-year-old man with congenital fronto-orbital lymphaticovenous malformation, refractory to endovascular techniques, which was later successfully ablated by a direct endoscopic-assisted orbital approach.