Despite high-dose corticosteroids and pulse methylprednisolone, a 55-year-old lady with recurrent polychondritis had increasingly increased right retro-orbital tumor invading the optic nerve, followed by left retrobulbar infiltrating lesions. Histopathologic examinations revealed thick collagen fibers with few inflammatory cells, supporting the diagnosis of idiopathic sclerosing orbital inflammation. This condition has been identified as a discrete entity with different clinical characteristics and associated rheumatologic disorders, necessitating more targeted diagnostic techniques and therapy regimens. In conclusion, we show a rare ocular symptom of recurrent polychondritis and underline the significance of repeated radiological and pathological assessments in such patients with exophthalmos.