We present four patients with sarcoidosis of the anterior visual pathw
ays. The first patient presented with unilateral visual loss, a mass l
esion at the optic nerve head, and an enlarged orbital optic nerve. Th
e second patient presented with bilateral progressive painless visual
loss, associated with optic nerve pallor and visual field loss. In the
se two patients, optic nerve biopsy was diagnostic of sarcoidosis. The
third patient developed optic nerve and chiasmal involvement after sa
rcoidosis was established by lacrimal gland biopsy. The fourth patient
had optic nerve, pulmonary, and lymph node involvement with sarcoidos
is. A conjunctival and lung biopsy were diagnostic. Computed tomograph
y and magnetic resonance imaging have greatly facilitated diagnosis of
sarcoidosis of the anterior visual pathways. Sarcoidosis of the anter
ior visual pathways may occur alone or in association with other ocula
r or systemic manifestations. A conjunctival or lacrimal gland biopsy
may be preferable as the initial diagnostic approach. Treatment of pat
ients with this condition may require systemic immunosuppression, in a
ddition to corticosteroids, to prevent permanent visual loss.