Purpose To report the clinical manifestations of 199 patients with suspected conjunctival lymphoma, the associations between these features and the pathological diagnoses, and the prognosis of conjunctival lesions during long-term follow-up.

Methods We conducted a retrospective chart review of 199 patients who underwent conjunctival biopsy on suspicion of conjunctival lymphoproliferative disease between January 2008 and June 2015. We focused on slit-lamp findings in the conjunctiva and the pathological diagnoses.

Results In total, 261 specimens of 199 patients were included in this study. The median age of patients was 42 years (range, 16–87 years), and those over 60 years of age constituted 17.1% of all patients. The proportion finally diagnosed with mucosa-associated lymphoid tissue (MALT) lymphomas was 58.2%. In these patients, the most common slit-lamp findings were the ‘salmon patch’ appearance (73.7%), followed by a follicular appearance (14.5%) and a nodular or subconjunctival mass (6.6%). Bilateral ocular manifestations were more common in patients with disease with the follicular appearance, as compared with patients with the salmon-patch appearance.

Conclusion Conjunctival MALT lymphoma presents in various ways, not only with the salmon-patch appearance. Therefore, biopsy should be considered if suspicion is raised, even though the conjunctival lesion does not exhibit the typical appearance of MALT lymphoma. In cases of follicular lesions responding poorly to topical steroids, a conjunctival MALT lymphoma may be suspected, given that chronic inflammation may precede neoplasia in patients with extranodal marginal zone lymphoma.