Ophthalmic involvement in the rheumatic diseases may take the form of a broad range of orbital manifestations. Indeed, orbital involvement can be the sole or major feature of some diseases such as Wegener's granulomatosis, in which orbital masses and proptosis are common. These complications can have cosmetic implications, but can also lead to sight-threatening disease, if severe corneal exposure occurs, of if compressive optic neuropathy develops. This article discusses the clinical features of orbital involvement in the presence of systemic rheumatic disease, as well as current and emerging therapies for its management. © 2011 Bentham Science Publishers Ltd.