Compressive optic neuropathy with acute or chronic vision loss has been ass
ociated with various skull base tumors, aneurysms, Graves disease, trauma,
and, less commonly, fibrous dysplasia and osteopetrosis. The authors presen
t a case of acute visual deterioration in a 25-year-old woman who had massi
ve calvarial hypertrophy with optic canal stenosis secondary to renal osteo
dystrophy (uremic leontiasis ossea [ULO]; bighead disease). Significant Vis
ual field restoration was achieved with high-dose corticosteroids followed
by optic nerve decompression. This is the first case report of cranial neur
opathy associated with ULO.