Objective: The purpose of the study was to report an atypical chorioretinal manifestation concomitant with severe scleritis in a patient diagnosed with relapsing polychondritis.

Methods: A 53-year-old man with a 6-month history of recurrent bilateral auricular cartilage inflammation was admitted to our hospital. Clinical and pathologic examinations of the auricular cartilage led to the diagnosis of relapsing polychondritis.

Results: Ophthalmologic examination revealed a localized elevation in the superonasal quadrant of the left fundus, where the scleritis was more severe than in other regions. B-mode ultrasonography revealed choroidal thickening and subretinal lesions in this area, along with a white retinal lesion identified during fundus examination. Further investigation using optical coherence tomography unveiled subretinal and retinal lesions in the same region, with dispersed cells into the vitreous cavity in a fountain-like pattern. After treatment with systemic corticosteroids and cyclophosphamide, there was an improvement in scleritis, conjunctivitis, and retinal vasculitis; however, the white retinal lesion progressed to retinal atrophy. Concurrently, a decrease in the number of vitreous cells emanating from the area of fountain-like leakage was noted.

Conclusions: Retinal manifestations of relapsing polychondritis are underrepresented in the literature. This report presents a unique case of relapsing polychondritis with a localized retinal manifestation observed on optical coherence tomography imaging, thereby broadening our understanding of the potential localized retinal presentations associated with this disease.