Purpose The purpose of this study was to report the clinicopathological features and management of the first case of bilateral synchronous conjunctival myxoma. Methods This study was a case report and literature review. Results A 66-year-old Chinese male with past ocular history of uncomplicated bilateral phacoemulsification and intraocular lens (IOLs) 3 years ago prior to presentation presented with bilateral red and swollen conjunctiva for over a year. On examination his corrected distance visual acuity (CDVA) was 25/20 in the right eye 20/20 in the left eye. Slit lamp examination revealed swollen temporal conjunctiva bilaterally which appeared as painless, well-circumscribed, salmon-pink, fleshy patches. The lesion in the right eye was subsequently excised, followed by excision of the lesion in the left eye at 3-week interval. Microscopically, histopathological examination of both excised specimens revealed hypocellular conjunctival mucosa covered by non-dysplastic epithelium, with presence of myxoid degeneration in the subepithelial stroma and immunostaining findings consistent with conjunctival myxoma. At his latest follow-up at 24 months, there were no recurrences of the conjunctival masses and the CDVA was the same as preoperatively.