A patient with systemic sclerosis (scleroderma) had diplopia. On the basis of results from ophthalmologic examination, muscle enzyme studies, and muscle biopsy, he was found to have ocular myositis, as well as an asymptomatic proximal myositis. The diplopia and the myopathy resolved promptly with corticosteroid therapy. To our knowledge, this report represents the first documentation of this complication in systemic sclerosis. We stress the therapeutic importance of recognition of inflammatory myopathy in systemic sclerosis and review the literature concerning the ocular manifestations of systemic sclerosis.