Objective To summarize the laboratory, pathologic and imaging characteristics of patients with autoimmune pancreatitis (AIP) retrospectively for early diagnosis and treatment. Methods Eleven AIP patients were selected in our hospital from 2007 to 2009. Laboratory parameters including complete blood count, tumor markers, hemodiastase and autoantibodies were tested. Abdominal ultrasonography and computerized tomography (CT) scanning were also performed. Results Five patients had elevated hemodiastase and liver enzymes, four patients had hyperbilirubinemia and three patients had hyperglobulinemia. Increased serum CA199 levels (mean 78.4 U/ml) were found in six patients. All of the 11 patients had enlarged pancreas on imaging. Four cases were treated with three months of drug therapy. After the treatment, the pancreas volumereturned to normal size, pancreatic duct and bile duct became normal in size. Seven cases received surgical operation. Pathology examination showed fibrous tissue and folliculus lymphaticus formation in the pancreas,with a large number of lymphocytes and plasma cells infiltration. Two of seven cases were diagnosed with Sj(o)gren's syndrome and rheumatoid arthritis after being followed-up for six to twelve months post-operation.Their symptoms relieved after corticosteroid and immunosuppressive therapy. Conclusion AIP is a special type of chronic pancreatitiswith characteristic laboratory, imaging and histological features. Glucocorticoid and immunosuppressive agents therapy are effective for AIP.