Abstract: Pancreatolithiasis is a rare benign pancreatic disease. In recent years, the detection rate increases with the popularization of imaging technology. Most patients with pancreatolithiasis also have chronic pancreatitis at the same time, which can lead to internal and external secretion disorder and even induce pancreatic cancer. Thus, it should be treated timely once diagnosed. This article reviews the research achievements about the pathogenesis and treatment in recent years, in order to provide reference for clinical practice.