Cogan's syndrome is a rare inflammatory disorder, classically characterized by interstitial keratitis and sensorineural hearing loss. Inner ear disease may result in deafness. In some patients, it may also be accompanied by systemic vasculitis. This syndrome is rare and very few cases have been reported since Cogan's first characterization in 1945. We report a rare case of a patient with typical Cogan syndrome who developed hepatic vasculitis with hepatic infarction mimicking multiple liver abscesses. Diagnosis of CS is often missed or delayed due to its rarity, the nonspecific clinical signs at onset, and the lack of a confirmatory diagnostic test. Rheumatologists should be aware of this entity and its possible systemic manifestations. The present case is unique because of the severity and involved area of the vasculitis.