Abstract Background Approximately 2 per cent of pancreatic masses resected for suspected malignancy are found instead to be a form of chronic pancreatitis defined by a characteristic lymphoplasmacytic infiltrate. This condition is now commonly classified as ‘autoimmune pancreatitis’. Methods A literature review of autoimmune pancreatitis was performed using Medline and PubMed. The reference lists of identified articles were searched for further relevant publications. Results Patients are predominantly 55-65 years old and present with obstructive jaundice, abdominal pain and weight loss. Imaging may show a mass of malignant appearance or pancreatobiliary tree strictures precipitating surgical exploration. Raised serum levels of IgG4 and specific autoantibodies, when combined with particular radiological features and a biopsy negative for malignancy, enable a preoperative diagnosis and successful treatment with steroids. Conclusion Autoimmune pancreatitis is not uncommon and steroid treatment can effect a dramatic improvement. Care is needed to ensure that pancreatic cancer is not misdiagnosed.