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Spontaneous and complete regression of malignant neoplasms is extremely unusual. To our knowledge, this case report is the first description of spontaneous regression of an extranodal malignant lymphoma occurring in the conjunctiva and orbit. A 40-year-old woman noticed a pink conjunctival mass at the medial aspect of her left eye that had been present for 3 weeks. She presented on May 5, 2003. Ophthalmologic examination showed a salmon-colored mass along the lateral side of the caruncle. CT revealed a mass in the medial orbit. Surgical biopsy exhibited a malignant lymphoma, diffuse large B-cell type. After biopsy, the tumor spontaneously decreased in size and completely disappeared in 5 weeks. At 6 months’ follow-up, the tumor had not recurred.