Liver, biliary tract and pancreas are the most frequently involved parts of IGG4-related diseases (IgG4-RD). Because of its non-specific clinical features, the definite diagnosis of IgG4-RD has become the key point and difficulty in the diagnosis and treatment of this kind of diseases. It is easy to cause missed diagnosis and misdiagnosis of this kind of diseases in clinical practice and delay the prescription of treatment. In recent years, the emergence of some highly sensitive biomarkers and the progress in the pathogenesis of IgG4-RD have provided a new theoretical basis for early diagnosis and the selection of therapeutic targets. This article reviews the current status of diagnosis and treatment and research progress of IGG4-related hepatobiliary and pancreatic diseases, in order to improve surgeons' systematic understanding of such diseases and prospect the possible research hotspots in the future.