A case of primary systemic amyloidosis with bilateral asymmetric involvement of the vitreous was followed up throughout a 45-month span, during which two vitrectomy procedures were done on one eye. The progressive nature of this disease was documented by serial fluorescein angiograms and fundus photography. The amyloid seemed to be derived from the retina, at small multifocal areas over arterioles and venules that were otherwise clinically and angiographically normal in appearance. Follow-up studies suggested that these focal deposits were actually preretinal, since they disappeared after posterior vitrectomy. Peripheral neovascularization was visible in this case, which has not previously been reported. Amyloidosis of the vitreous can mimic numerous other diseases. Vitrectomy can be effective in restoring visual acuity, although recurrence may be rapid. The diagnosis is readily made by pathologic examination of the vitreous aspirate.