We describe two cases of type 2 autoimmune pancreatitis (AIP). A 39-year-old man presented to our hospital with complaints of epigastric and back pain. Pancreatic enzyme levels were elevated, but serum levels of immunoglobulins G and G4 (IgG and IgG4) were normal. Computed tomography (CT) showed diffuse pancreatic enlargement, and endoscopic retrograde pancreatography revealed diffuse narrowing of the pancreatic duct. Endoscopic ultrasound-guided fine-needle aspiration (EUS-FNA) revealed granulocytic epithelial lesions and very few IgG4-positive cells. Colonoscopy revealed ulcerative colitis. Type 2 AIP was diagnosed, and 5-aminosalicylic acid (5-ASA) and prednisolone were administered. The clinical course has since been favorable, and the prednisolone dose is currently being reduced. A 47-year-old woman presented to our hospital with complaints of bloody stools. Colonoscopy revealed ulcerative colitis. CT depicted diffuse pancreatic enlargement with a capsule-like rim. Pancreatic enzyme levels were elevated, but serum levels of IgG and IgG4 were normal. On magnetic resonance cholangiopancreatography, the pancreatic duct could not be delineated. No pathological findings of type 2 AIP were obtained on EUS-FNA. Type 2 AIP was suspected, and 5-ASA and steroid enemas were administered. To date, recurrence has not been observed, and 5-ASA management continues. The two cases differed with regard to sex of patient, clinical course, pathological findings, and treatment.