Retrospective review of 33 patients followed for up to 14 years showed that no signs, symptoms, or histopathologic classification were able to correlate with the long-term fate of the patient. In some cases the histologic pictures were diagnosed differently by different pathologists, pointing up the difficulty encountered in differentiating benign from malignant tumor. Among the interesting clinical findings were: (a) all eight cases of bilateral involvement of the eye or orbital involvement proved to be or developed later systemic lymphomas; (b) one half of the systemic cases (7 out of 15) had ophthalmic involvement from the start; (c) five out of six cases of the medial conjunctival involvement were found to be ultimately malignant. A long-term follow-up and the fate of the patient has to be the final arbiter as to whether the lesion was benign or malignant in a significant number of cases.