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Cogan’s syndrome (CS) is a chronic inflammatory disorder that most commonly affects young adults. Major clinical features are interstitial keratitis and vestibuloauditory dysfunction. Associations between CS and systemic vasculitis as well as aortitis also exist. The present report is the first case in the literature of pregnancy associated with Cogan syndrome, which posed a therapeutic challenge. There was a relapse of the ocular symptoms only during the first trimester of pregnancy, but the pregnancy was otherwise uneventful. The relevant literature is reviewed both with regard to the relationship of CS to pregnancy and the therapeutic approach in this situation.