Chronic lupus-like illness, characterized by arthralgia, cutaneous manifestations of lupus erythematosus, and photosensitivity, occurred in two mothers of boys with chronic granulomatous disease; one mother also had Raynaud's phenomenon, pleuritis, and unexplained fevers, and both had recurrent stomatitis. Both mothers were carriers with a population of defective leukocytes demonstrable in vitro. Neither had hypergammaglobulinemia or positive tests for antinuclear antibodies, lupus erythematosus cells, or rheumatoid factors. The occurrence of lupus, particularly discoid lupus, in carriers of chronic granulomatous disease has been sporadically mentioned and appears to be more frequent than would be expected from a chance occurrence. The association of lupus-like illness with this partial immune defect may be pertinent to the hypothesis that lupus erythematosus results in some way from an infectious process.