The article contains the description of a clinical case of the patient P. of 59 years with autoimmune pancreatitis (AIP) associated with a sclerousis cholangitis (SC), and the analysis of literature on this problem. The patient was operated (cholecystenterostomy, enteroenterostomy) for pancreatic cancer. The pancreatic cancer was excluded. Overlap syndrome (primary biliary cirrhosis/autoimmune hepatitis), pseudotumorosis pancreatitis were excluded during the period of monitoring in hospitals of Moscow. In our clinic the diagnosis AIP is established on the basis of Mayo's criterion (HISORt: Histology, Imaging, Serology, Other organ involvement, Response to corticosteroid therapy). The increased level of immunoglobulin G (IgG) by 3 times (46,2 g/l, norm--6.58-18.37 g/l), IgG4 by 25 times (49.5 g/I, norm--0.33-2.01 g/l) was revealed. MRI of abdominal organs showed diffusion increase pancreas with a peripheral hypointensive rim, local stenosis of the Major pancreatic duct in the head pancreas. Immune histochemical study surgical biopsy of pancreas showed large number of IgG-positive plasma cells. Glucocorticosteroids (GCS, prednisolone 40 mg/d), ursodeoxycholic acid 1000 mg/d, creon 120000 ed/d promoted improvement of clinical picture AIP and decrease IgG4 (to 6.4 g/I). After 12 months IgG4 wasn't defined. Control MRI revealed dilatation small biliary ducts in 8, 5, 4 liver segments and thickening wall common bile duct (to 2 mm) at large distance. The peculiarity of the case--the 15-year follow-up of patients with AlP who had inappropriate surgery. Late diagnosis and delayed treatment GCS adversely affected the course of the disease, which led to the development of secondary biliary cirrhosis.