Abstract: Objective To search the best evidence so far, and to provide the most powerful evidence for the diagnosis and treatment of a suspected immunoglobulin G4-related autoimmune pancreatitis（IgG4-RAIP） in our hospital. Methods Searching for systematic evaluation, clinical guidelines, cross-sectional studies, medical record reports, literature review, expert recommendations, etc from Embase database （1995-2019）, Medline database, Cochrane Library （Central database） （1995-2019）, Uptodate database, Wanfang database （1995-2019）, China knowledge net CNKI （1995-2019）; evaluating and grading the literature evidences. Results The retrieved evidences show that focal IgG4-related pancreatitis is easily misdiagnosed as pancreatic head cancer, and the differentiation between them depends on pancreas biopsy. Since surgical resection is the first choice for the treatment of pancreatic cancer and glucocorticoid therapy is the first choice for IgG4-related pancreatitis, it is particularly important to perform pancreatic biopsy in patients with pancreatic cancer before operation or in patients with suspected IgG4-related pancreatitis. Conclusions Pancreas biopsy and serum immunological examination have high specificity and sensitivity in the diagnosis of IgG4-related pancreatitis. Glucocorticoid is a specific drug for the treatment of IgG4-related pancreatitis.