Background and Aims Autoimmune pancreatitis (AIP) is a rare form pancreatitis caused by an autoimmune process with an incidence of about 10.1/100 000, and a positive response to steroids. However, it is difficult to distinguish focal AIP from pancreatic cancer, and there have been cases of misdiagnosis as pancreatic cancer leading to surgical treatment. Currently, the pathogenesis of AIP is unclear, and there is still a lack of relevant research. This article reports the diagnosis and treatment process of two cases of type 1 AIP recently admitted to our center, and additionally reviews the relevant literature in order to provide a useful reference for clinical work.Methods The clinical data of two patients with type 1 AIP admitted to the Department of Hepatobiliary and Pancreatic Surgery at the Third Xiangya Hospital of Central South University were retrospectively analyzed, combined with review of the relevant literature. The clinical characteristics and treatment decisions of this disease were analyzed and summarized.Results Both patients were male and presented with obstructive jaundice. Imaging examinations showed a mass in the pancreatic head, which was difficult to distinguish between inflammation and tumor. Case 1 had significantly elevated blood IgG4 level and extrapancreatic organ involvement, and was diagnosed with type 1 AIP. The patient's condition improved after steroid treatment. Case 2 was relatively atypical, with no blood IgG4 elevation or extrapancreatic organ involvement. Pathological examination of the ultrasound-guided fine-needle aspiration biopsy showed chronic inflammation, and further diagnosis and treatment were performed with laparoscopic pancreaticoduodenectomy. During the surgery, the pancreatic head was found to be significantly enlarged, hard in texture, and closely adhered to the surrounding tissues. Postoperative pathology revealed typical lymphoplasmacytic sclerosing pancreatitis (LPSP), and the patient was diagnosed with type 1 AIP. The patient's condition improved after steroid treatment.Conclusion For patients with pancreatic mass suspicious for pancreatic malignancy, the possibility of AIP should always be considered, and immunological markers and other examinations should be perfected. Typical cases are generally easy to diagnose, while atypical cases may require surgical resection before a final diagnosis can be made. For middle-aged and elderly men with normal or slightly elevated tumor markers and negative puncture pathology, the possibility of benign lesions is high, and postoperative pathology should be closely monitored. If the diagnosis of AIP is made, patients should be informed to receive standardized medical treatment as soon as possible.