PURPOSE: This report describes a rare and early extraintestinal manifestation of ulcerative colitis (UC), presenting as recurrent, bilateral, sequential neuroretinitis (NR). To our knowledge, this is the first documented case of UC-associated NR successfully treated with a combination of cyclophosphamide and adalimumab. METHODS: Retrospectively, multimodal imaging and medical history were obtained. RESULTS: A 54-year-old male presented with central vision loss in the left eye. Fluorescein angiography confirmed sectoral papillitis, and optical coherence tomography revealed intraretinal fluid originating from the optic disc and subfoveal neuroepithelial detachment, consistent with NR. Although the patient initially responded to oral prednisone, optic disc edema progressed to optic atrophy. UC was diagnosed one month later and managed with a tapering course of prednisone in combination with mesalazine as maintenance treatment. Nine months later, the patient developed papillitis in the contralateral eye, which responded to oral prednisone. Azathioprine was introduced for relapse prevention. However, a subsequent NR episode occurred in the right eye two months later, requiring three intravenous pulses of methylprednisolone. Due to worsening visual field and persistent optic disc inflammation, confirmed by fundoscopic examination and magnetic resonance imaging, monthly cyclophosphamide therapy was initiated. After four doses, NR resolved. Given the presumed extraintestinal nature of the ocular involvement, adalimumab was introduced, resulting in sustained remission without further recurrences. CONCLUSION: This case highlights that inflammatory bowel disease may manifest initially with ophthalmological findings like NR. Immunosuppressive therapy-including cyclophosphamide- may be necessary to control inflammation, and long-term biologic therapy can play a key role in preventing relapses.