Type I autoimmune pancreatitis (AIP) typically presents in older men, with a mean age of onset in the fifth decade of life. This patient has evidence of type 1 AIP based on the presence of three diagnostic criteria: imaging features (focal pancreatic enlargement with a featureless rim and a nondilated pancreatic duct), increased serum IgG4 level, and extrapancreatic organ involvement (sclerosing cholangitis or IgG4-associated cholangitis). Almost all patients (>90%) enter clinical remission in response to glucocorticoids, but relapse is common