BACKGROUND Eosinophilic angiocentric fibrosis (EAF) is an extremely rare disease with characteristic histopathological findings of fibrotic onion-skin appearance and eosinophils. The lesion primarily affects the nasal cavity, paranasal sinus, and orbit. Although there have been approximately 78 cases of EAF reported in the literature to date, no cases of EAF in the eyelid conjunctiva have ever been reported. CASE REPORT Herein, we describe the case of a 55-year-old Japanese woman with a history of eosinophilic sinusitis and EAF in bilateral upper eyelid conjunctivas who underwent surgical resection of the affected tissue. Histopathological examination revealed collagen bundles winding around the vessels in an onion-skin pattern, and the presence of eosinophils, lymphocytes, and plasma cells. CONCLUSIONS We describe the first reported case of EAF in bilateral upper eyelid conjunctivas. It can be successfully treated by surgical resection, and with no recurrence within 6 months postoperatively.