The deposition of amyloid protein in orbital tissue is called orbital amyloidosis. Orbital amyloidosis is a rare condition that usually affects older patients. Although the disease is slowly progressive, it rarely involves the optic nerve or threatens vision. We report a case of orbital amyloidosis that initially appeared as impressive eyelid pigmentation and blepharoptosis and then progressed to systemic amyloidosis. The orbital lesion induced compressive optic neuropathy. Surgical management enabled partial but not full recovery of vision.