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To report a case of bilateral peripheral corneal thinning with an appearance of Fuchs superficial marginal keratitis in the right eye and Terrien marginal degeneration in the left eye.Interventional case report.A 28-year-old woman presented with bilateral corneal thinning and a pseudopterygium in the right eye and peripheral corneal thinning in the left eye. Laboratory examinations were negative. Over the next several years, the patient experienced periodic episodes of bilateral ocular redness and discomfort associated with gradual corneal thinning. However, other signs of progression were different in each eye. Pseudopterygium growth was observed in the right eye, consistent with Fuchs superficial marginal keratitis. Progressive lipid deposition at the leading edge of corneal thinning was observed in the left eye, consistent with Terrien marginal degeneration. Chronic topical corticosteroid therapy was initiated with little, if any, subsequent disease progression. A perinuclear antineutrophil cytoplasmic antibody (p-ANCA)-positive vasculitis was subsequently diagnosed.Fuchs superficial marginal keratitis and Terrien marginal degeneration may represent different clinical features of the same disease process. Laboratory investigation for underlying systemic disease may be warranted in patients with the appearance of either disorder. Chronic antiinflammatory therapy may be effective in limiting the progression of corneal thinning in these diseases.