A woman developed headaches, transient visual obscurations, anosmia, a
nd decreased visual acuity. Ocular examination showed bilateral pulsat
ile proptosis and disc edema with choroidal folds. Standardized ophtha
lmic echography showed absence of bony orbital roofs, prominent dural
pulsations, direct apposition of brain parenchyma and orbital tissues,
and echographic signs suggesting bilateral optic nerve compression. C
T and MRI showed a large defect in the floor of the anterior cranial f
ossa. The cribriform plate, both orbital roofs, and sphenoid bones wer
e displaced by a large basal encephalocele. Clinical improvement follo
wed reconstruction of the anterior cranial fossa and decompression of
both optic nerves.