A 39-year-old man with systemic lupus erythematosus (SLE) experienced three episodes of acute orbital inflammation, a primary feature of each being a markedly enlarged extraocular muscle (myositis). The muscles of the upper trunk were concomitantly inflamed, and full-thickness skin and muscle biopsy specimens demonstrated an inflammatory myositis. The case demonstrates that orbital proptosis and extraocular myositis can be features of SLE.