The development of multiple, round or oval, subtle, red patches at the level of the pigment epithelium in the posterior ocular fundus and their striking early hyperfluorescence angiographically are characteristic features of the bilateral diffuse uveal melanocytic proliferation syndrome. They may be accompanied by severe visual loss and may antedate the appearance of multiple melanocytic tumors, retinal detachment, and cataract in these patients with occult systemic carcinomas. These hyperfluorescent patches are caused by focal damage to the pigment epithelium overlying an intact choriocapillaris and diffuse benign nonpigmented uveal melanocytic infiltration of the outer choroid. We suggest that outer retinal damage may not be primarily caused by melanocytic proliferation, but rather by toxic and immune factors generated by interaction between a systemic carcinoma and congenital melanocytosis of the uveal tract. We report the longest survivor of this disorder to date (102 months).