A 62-year-old male was referred to our hospital because of liver dysfunction, diffuse pancreatic swelling, and trachelophyma. At admission, the patient was free of pain. Physical examination showed enlarged and palpable bilateral submandibular masses, but no palpable mass or organomegaly in the abdomen. Laboratory findings were as follows: total protein 90 g/L with y-globulin of 37.3% (33 g/L), total bilirubin 4 mg/L, aspartate aminotransferase 39 IU/L, alanine aminotransferase 67 IU/L, y-glutamyl transpeptidase 1 647 IU/L, and amylase 135 IU/L. Autoanti-bodies were negative, and tumor markers were within the normal range. Serum IgG4 level was markedly elevated (18 900 mg/L). Computed tomography (CT) showed diffuse swelling of the pancreas and dilatation of both common and intra-hepatic bile ducts. Endoscopic retrograde pancreatography (ERP) revealed diffuse irregular and narrow main pancreatic duct and stenosis of the lower common bile duct. Biopsy specimens from the pancreas, salivary gland and liver showed marked periductal IgG4-positive plasma cell infiltration with fibrosis. We considered this patient to be autoimmune pancreatitis (AIP) with fibrosderosis of the salivary gland and biliary tract, prescribed prednisolone at an initial dose of 40 mg/d. Three months later, the laboratory data improved almost to normal. Abdominal CT reflected prominent improvement in the pancreatic lesion. Swelling of the salivary gland also improved. At present, the patient is on 10 mg/d of prednisolone without recurrence of the pancreatitis. We present here a case of AIP with fibrosderosis of salivary gland and biliary tract. A 62-year-old male was referred to our hospital because of liver dysfunction, diffuse pancreatic swelling, and trachelophyma. At admission, the patient was free of pain. Physical examination showed enlarged and palpable bilateral submandibular masses, but no palpable mass or organomegaly in the abdomen. Laboratory findings were as follows: total protein 90 g / L with y-globulin of 37.3% (33 g / L), total bilirubin 4 mg / L, aspartate aminotransferase 39 IU / L, alanine aminotransferase 67 IU / L , y-glutamyl transpeptidase 1 647 IU / L, and amylase 135 IU / L. Autoanti-bodies were negative, and tumor markers were within the normal range. Serum IgG4 level was markedly elevated (18 900 mg / CT) showed diffuse swelling of the pancreas and dilatation of both common and intra-hepatic bile ducts. Endoscopic retrograde pancreatography (ERP) revealed diffuse irregular and narrow main pancreatic duct and stenosis of the lower common bile duct. Biopsy specimens from the pancreas, salivar We considered this patient to be autoimmune pancreatitis (AIP) with fibrosderosis of the salivary gland and biliary tract, prescribed prednisolone at an initial dose of 40 mg / d. Three At present, the patient is on 10 mg / d of prednisolone without recurrence of the pancreatitis. We present here a case of AIP with fibrosderosis of salivary gland and biliary tract.