Lesions of the caruncle are very rare. Most caruncular lesions are benign, though there were still few reported malignant lesions. Ocular adnexal lymphoproliferative disease (OALD), one of the differential diagnosis of caruncular lesion, is seldom seen in young pa-tient. In this article, we report a 16-year-old boy with recurrent caruncular atypical lymphoid proliferation. We discuss the epidemiology, clinical features, images, histomorphology, and treatment between ocular adnexal lymphomas (OAL) and reactive lymphoid hyperplasia (RLH).