IntroductionCogan's Syndrome is typically characterised by a non syphilitic interstitial keratitis (IK), with or without conjunctivitis, iritis or subconjunctival bleeding and progressive sensorineural hearing loss within two years of ocular signs. Atypical ocular manifestations include episcleritis, scleritis, posterior scleritis, retinal artery occlusion, choroiditis, retinal vasculitis, and optic disc oedema. We report a case of Cogan's syndrome in with recurrent cystoid macular oedema was the main feature. Case Presentation A patient was diagnosed with Cogan's syndrome nearly 2 years after first presentation. He had cystoid macular edema which failed to respond initially to steroid, methotrexate and azothiaprine however resolved after treatment with mycophenolate mofetil.Conclusion:Cogan's syndrome is rare and presents a challenge in terms of diagnosis and treatment. Recurrent cystoid macular oedema is a unique finding in this condition and can be difficult to control. Multidisciplinary management of this multisystem disorder is vital.