Scleroderma en coup de sabre, a variant of localized scleroderma, is a disorder of unknown origin characterized by fibrosis of connective tissue. Rare complications of scleroderma en coup de sabre are orbital and intracerebral involvement. We describe a patient with scleroderma en coup de sabre in whom intracerebral and orbital lesions developed after 2 decades of disease duration. Clinically, she had epilepsy, impaired vision, and retro-ocular pain of the affected eye. A 12-month course of interferon-gamma stopped progression of visual symptoms caused by orbital fibrous tissue. To our knowledge, this is the first patient with scleroderma en coup de sabre complicated by orbital involvement who was successfully treated with interferon-gamma.