The purpose of this study is to characterize the chronic liver disease (CLD) that may be associated with familial Mediterranean fever (FMF). Twenty-seven patients (mean age, 48 ± 18 years; F/M, 16:11) with FMF who were referred for assessment of CLD were studied. Data regarding FMF and CLD were obtained from patient medical files. Liver biopsy was performed in 21 of 27 patients and deferred in six (cirrhotic coagulopathy in five and one who improved after colchicine dose reduction). Patients with FMF and non-alcoholic fatty liver disease (NAFLD) were compared to matched controls from a cohort of 150 patients with NAFLD per liver biopsy but without FMF. The mean Tel Hashomer severity score was 1.7 ± 0.9. The mean daily dose of colchicine was 1.4 ± 0.4 mg over a mean duration of 21 years ± 10. Seven of ten patients who underwent mutation analysis for FMF were homozygous for M694V. In 15 patients, there was evidence of NAFLD: five with "simple" steatosis, three with non-alcoholic steatohepatitis (NASH), and seven with NASH-cirrhosis. An additional five patients had "cryptogenic" cirrhosis, which in most patients represents the end result of unrecognized NASH, and one had normal liver tissue. Comparing FMF patients with NAFLD to matched controls with NAFLD did not reveal excess of metabolic syndrome in FMF patients. Of our FMF patients, 74% had evidence of NAFLD, 75% of which with severe manifestation. The extremely high proportion of NAFLD in our cohort of FMF patients without overt metabolic syndrome may indicate an unappreciated novel association between FMF and NAFLD.