This study aimed to clarify the natural history of TRPV6-related pancreatitis and the impact of pancreas-specific deletion of Trpv6 on pancreatitis in mice.<h4>Methods</h4>Clinical information of the patients carrying functionally impaired TRPV6 variants, defined by Ca<sup>2+</sup> imaging and minigene assays, was collected from six international centers. This evidence concerns the gene TRPV6 and pancreatitis.