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Fatty liver disease is an increasingly common indication for liver transplantation. Because of its prevalence, it may coexist with other causes of chronic liver disease, many of which may carry implications for treatment or follow-up of patients or their families. An increased degree of suspicion may be required to detect concurrent liver disease. We report a case of a 71-year-old man who underwent liver transplantation for nonalcoholic steatohepatitis. The explanted liver showed evidence of coexisting α1-antitrypsin deficiency disorder. The clinicopathologic features and epidemiology of fatty liver disease and α1-antitrypsin deficiency disorder are discussed, along with other possible coexisting disease.