Purpose.To describe a 30-year-old man with bilateral but asymmetric anterior keratoconus and unilateral cornea guttata in the eye with more advanced keratoconus.Method.Case report.Results.The patient's keratoconus was diagnosed three or four years earlier. The keratoconus was confirmed by slit-lamp examination, keratometry, and computer-assisted topographic analysis. Cornea guttata were confirmed by clinical examination and specular microscopy.Conclusion.This is a rare case of unilateral cornea guttata associated with asymmetric keratoconus, showing severe guttate change only in the eye with more advanced keratoconus.