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Abstract Background  To report on conjunctival nodules as an unusual manifestation of Vogt-Koyanagi-Harada disease. Methods  A 24-year-old woman presented with a two-month history of bilateral conjunctival injection and gradually decreased vision. Ophthalmological examinations revealed bilateral granulomatous uveitis and bulbar conjunctival nodules, and a biopsy of the conjunctival nodules was performed. Results  The biopsy specimens showed noncaseating granulomas. The major components of the infiltrating lymphocytes were CD8-positive T cells. Topical corticosteroid therapy reduced the anterior segment inflammation, and the conjunctival nodules disappeared within a week. Afterwards, the patient demonstrated bilateral retinal detachments, sunset glow fundus and alopecia, and, therefore, was diagnosed to have Vogt-Koyanagi-Harada disease four months after the first symptoms. Conclusions  Conjunctival nodules may represent the primary manifestation of Vogt-Koyanagi-Harada disease. [ABSTRACT FROM AUTHOR]