PURPOSE To report a patient with severe and progressive bilateral deafness who suffered recurrent episodes of diffuse lamellar keratitis (DLK) in both eyes > or = 4 months after undergoing uneventful LASIK for myopia. METHODS Four months after LASIK, the patient presented with blurred vision in the left eye. Best spectacle-corrected visual acuity was 0.9. Slit-lamp examination revealed aggregates of white cells at the paracentral and mid-peripheral interface without affecting the visual axis. Diffuse lamellar keratitis stage II was diagnosed and intensive corticosteroid treatment was started. Uncorrected visual acuity (UCVA) in the right eye was 1.2 with no signs of DLK. RESULTS One week after initiating corticosteroid treatment, UCVA improved (1.2) in the left eye. Diffuse lamellar keratitis was diagnosed in the left eye again at 9 months and in the right eye at 15 months after LASIK. The patient was later diagnosed with atypical Cogan's syndrome. CONCLUSIONS Recurrent and late-onset DLK may be due to other patient systemic conditions predisposing to interstitial keratitis, such as Cogan's syndrome.