A 4-month-old boy presented with the sudden onset of proptosis and dilated episcleral veins. CT revealed an enlarged superior ophthalmic vein and diffusely thickened extraocular muscles. MRI and angiography confirmed a dural middle meningeal--cavernous sinus fistula. Embolization was performed leading to successful resolution of the patient's signs and symptoms. Spontaneous atraumatic arteriovenous cavernous fistulae in infancy are extremely rare, but should be included in the differential diagnosis of infants with orbital congestion. Appropriate treatment leads to prevention of amblyopia, glaucoma, and anisometropia.
