We report a patient who developed keratitis and corneal edema during treatment with carbidopa-levodopa. The patient was a 34-year-old woman who complained of apraxic gait after anoxic brain damage caused by ventricular arrhythmia. She had difficulty in walking. While doses of droxidopa, flecainide acetate and amantadine HCl were kept at the same levels as before several weeks, administration of carbidopa-levodopa was gradually increased from an initial dose of 100 mg per day to 300 mg per day over a period of 10 days in an attempt to relieve her apraxic gait. Ten days after initiation of treatment with carbidopa-levodopa, her postural instability and unsteady gait slightly improved. However, after 8 days of treatment with carbidopa-levodopa, she complained of blurred vision. Ophthalmologic examination showed keratitis (corneal endothelitis) and corneal edema. After stopping carbidopa-levodopa, the keratitis dramatically improved. Cessation of the drug therapy resulted in a return of vision to normal levels by the 7th day. Although there had been no previous reports of corneal lesion caused by levodopa, we suspected that keratitis and corneal edema were associated with carbidopa-levodopa use, especially in combination treatment with flecainide acetate, and amantadine HCl. The process was reversible and presumably could have been prevented by a shorter term of medication.