The IgG4-related sclerosing disease is characterized by the presence of plasmatic IgG4 positive cells and T-lymphocytes infiltration in different organs. We herein report a case of cholestasis due to autoimmune cholangitis associated to IgG4 disease. A 40-year-old woman with a history of pruritus, anosmia, Sjögren's syndrome and diabetes, was referred for a pancreatic tumor. Alkaline phosphatase was 24-fold upper limit of normal (ULN), gamma-glutamyl transpeptidase 21-fold ULN, aspartate aminotransferase 3-fold ULN, alanine aminotransferase 2-fold ULN, cholesterol 408 mg/dL, bilirubin normal, gamma-globulin 3.92 g/dL, IgG4 4.6 g/L, antinuclear antibody positive (1/320), and antimitochondrial antibodies negative. Ultrasound scan (US) showed a mass in the pancreatic head and thickening of the gallbladder and the bile duct walls. Dilation and strictures of the main pancreatic duct and intrahepatic bile ducts were detected by MR cholangiopancreatography. Liver biopsy showed chronic inflammatory lesions, ductal damage (autoimmune cholangitis) (METAVIRA2, F2) and IgG4 bearing plasmatic cells. A cervical lymph node showed IgG4 bearing plasmatic cells. After 2 weeks of treatment with meprednisone, ursodeoxycholic acid and insulin, pruritus and anosmia disappeared. After eleven months of treatment imaging studies showed disappearance of the pancreatic tumor, atrophy of the body and the pancreatic tail and normal biochemical parameters, except for alkaline phosphatase 2-fold ULN. The final diagnosis of our patient was autoimmune hepatitis with cholangitis associated to IgG4 systemic diseases.