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Purpose: To describe a case with a growing erythematous conjunctival mass in the left eye, diagnosed as conjunctival myxoma. Observations: A 31-year-old lady had corrected visual acuity of 6/7.5 and normal intraocular pressure in both eyes. Congested left bulbar conjunctiva with one slightly elevated nodule at nasal area was noted for one year. Excisional biopsy was performed after failed treatment with topical eye drops. Pathology showed conjunctival myxoma with spindle- and stellate-shaped cells, which was a rare ocular surface neoplasia. There was no combined systemic disease found. Conclusions and importance: We described the clinical and pathological features of conjunctival myxoma. Systemic evaluation should be considered before and after surgical excision.