Centrilobular zonal necrosis (CZN) is not a pattern typically associated with autoimmune hepatitis (AIH). However, it has occasionally been reported that CZN occurs without the classic histologic features of AIH in patients with autoimmune abnormalities. In this study, we examined the clinicopathological features of 5 cases of CZN with autoimmune features not associated with classic AIH. The patients were 1 man and 4 women (24-82 years). Three patients had subjective symptoms (general malaise, arthralgia, and fever). All had antinuclear antibodies (1:40 to 1:1280). Liver biopsy showed CZN without any histologic features of classic AIH. Liver injury was sustained without medication in 4 cases, whereas it was spontaneously improved in 1 case. However, 2 months later, this patient was found to have recurrent liver dysfunction. Liver biopsy at the time of recurrence again showed CZN without the features of classic AIH. All patients were effectively treated with prednisone. Based on a review of a total of 17 cases of CZN with autoimmune features, including previously reported cases, the patients could be classified into 3 groups: cases without recurrence, cases with recurrent CZN, and cases with progression to classic AIH. Patients of the 2 former groups did not develop classic AIH during follow-up. Factors predictive of recurrence were younger age, being male, and high serum bilirubin or transaminase concentrations at first presentation (P < .05). This study suggested that CZN with autoimmune features corresponds to the early stage of classic AIH in some cases and might be a distinct type of autoimmune liver disease in others.