A case of intraorbital arteriovenous malformation presenting with visu
al loss, exophthalmos, and chemosis of the right eye is reported. Enha
nced computer tomography and magnetic resonance imaging showed extraoc
ular muscle enlargement and vascular abnormality in the right retrobul
bar space. Angiography revealed an abnormal intraorbital vascular stai
n with an extremely dilated right ophthalmic artery. Total removal of
the intraorbital contents was performed after unsuccessful endovascula
r and surgical treatment of arteriovenous malformation (AVM). Histopat
hological examination disclosed an AVM in the retrobulbar fatty tissue
with extension into the extraocular muscles and optic nerve.