Our discussion of adenocarcinomas arising from the ciliary and iris epithelium, based upon a case report and a review of the literature, attempts summarize what little is known about these tumors. In most instances, the tumors arise from previously healthy eyes in middle-aged people. There appears to be no known way to make the diagnosis preoperatively. As long as the tumor has not extended outside the globe at the time of removal, the prognosis is excellent. The morphology of the tumors is virtually identical to those arising from the retinal pigment epithelium, with anaplastic epithelioid cells often forming tubes, cords, and acinuslike structures. In a few cases, tumors with these traits have extended from the globe and have eventually resulted in the death of the patients; one of these cases unquestionably arose from the iris and ciliary body epithelium. Knowledge of these tumors is in a primitive stage, and much clinical and pathological information is needed in order to determine their casue, diagnostic clinical features, and proper treatment.