The idiopathic retinal vasculitis, aneurysms, and neuroretinitis (IRVAN) 
syndrome typically occurs in young patients and may produce multiple retinal 
macroaneurysms, neuroretinitis, and peripheral capillary nonperfusion. Optic 
disc edema has been described, but elevated intracranial pressure has not been 
previously documented. We report a case of a 12-year-old girl who presented with 
bilateral disc swelling and peripapillary hemorrhage. Brain magnetic resonance 
imaging (MRI) was normal, but lumbar puncture yielded an opening pressure of 360 
mm H2O with normal constituents. Fluorescein angiography delineated saccular 
aneurysms of the retinal arteriolar vasculature, and IRVAN syndrome was 
diagnosed. MR venography disclosed poor filling of both transverse venous 
sinuses. Acetazolamide treatment of 14 months did not alter the fundus findings. 
IRVAN syndrome may present initially with optic nerve swelling and elevated 
intracranial pressure with subsequent development of the characteristic retinal 
vascular abnormalities.