A 38-year-old man was referred to our hospital because of recurrent panuveitis in both eyes. Recurrent oral ulcer, folliculitis, and genital ulcer were noted as systemic complications. He had been treated with topical and systemic corticosteroids as needed for 5 years prior to presentation. On ophthalmologic examination, best corrective visual acuity (BCVA) was 20/60 in the right and 20/200 in the left eye, and intraocular pressures were 12 and 11 mmHg, respectively. Moderate cell infiltration into the anterior chamber (+2 cells), and diffuse vitritis (+1-2 cells) were observed in both eyes, but no definite retinal lesions were detected. However, fluorescein angiography (FA) showed severe dye leakage from extensive retinal vessels at the optic disc, macula and peripheral retina in both eyes. Spectral domain-optical coherence tomography (SD-OCT) revealed retinal cysts and disruption of ELM, EZ, and CIZ in the macular region of both eyes. BD was diagnosed based on the ocular features and systemic lesions. Since visual acuity in both eyes was being deteriorated by persistent ocular inflammation despite corticosteroid treatment, infliximab therapy was initiated. After 3 months of infliximab therapy, although foveal excavation was first recovered with disappearance of macular edema in both eyes, the disrupted outer retinal layers did not improve and BCVA remained unchanged. However, after 12 months of infliximab therapy, ELM and EZ were well defined in both eyes and BCVA improved to 20/40 in the right eye and 20/30 in the left eye. Finally, the CIZ became distinguishable after 24 months of infliximab therapy, and BCVA in both eyes was 20/25. Vasculitis indicated by dye leakage on FA remained only in peripheral retina, and ocular inflammatory attack did not recur in both eyes after the initiation of infliximab therapy.