Abstract: Immunoglobulin G (IgG)4-related pancreatitis, also named as type Ⅰ autoimmune pancreatitis, is recognized as a pancreatic manifestation of IgG4-related disease. It is very hard to differentiate it from pancreatic cancer due to similar clinical manifestations with pancreatic cancer. Diagnosis of IgG4-related pancreatitis mainly relies on pathological examination and imaging examination. Corticosteroids are the main treatment for IgG4-related pancreatitis, and the risk of relapse is still high with steroid taper or following withdrawal of steroids. Based on researches on IgG4-related pancreatitis and our clinical experience, the authors have analyzed and summarized pathogenesis, clinical manifestations, clinical diagnosis, differentiation from pancreatic cancer, clinical treatment of IgG4-related pancreatitis, and suggested that elucidation of its mechanism and induction of immunotherapy may improve its efficacy.