A 76-year-old patient developed cystic exudative retinal detachment with many yellowish subretinal precipitates and a large retinal pigment epithelial (RPE) tear in midperiphery of the right eye in the course of polyclonal hypergammaglobulinemia. The left eye showed localized RPE detachment in the posterior pole which gradually extended. Systemic steroid administration reduced the subretinal precipitates to some extent, but suspicion of malignancy led to enucleation of the right eye after unsuccessful diagnostic vitrectomy. A histopathological study revealed massive infiltration of the uvea with plasma cells and small lymphocytes but no abnormal cells. This is the first report to show the association of an RPE tear with reactive lymphoid hyperplasia of the uvea.