Corneal and scleral disorders related to inflammatory rheumatic diseases vary both in frequency and in severity. Sicca syndrome and its complications are the most common ocular manifestations and, together with episcleritis, can usually be managed by topical treatments. In contrast, the various forms of scleritis and peripheral ulcerative keratitis generally require systemic glucocorticoid therapy and the initiation or intensification of immunosuppressive treatment. Corneal and scleral manifestations are inaugural in a few patients with chronic inflammatory rheumatic disease. No direct information is available on the frequency of severe corneal and scleral involvement, which can only be estimated by extrapolating data from case-series or cohorts, many of which are historical. Similarly, given the absence of randomized controlled trials, treatment decisions must rely on clinical experience acquired in referral centers and on reports of small case-series studies. The rheumatologist and ophthalmologist must work closely together to ensure the prompt and optimal management of these potentially serious conditions.