Type 1 autoimmune pancreatitis (AIP) is an IgG-4–related systemic disease that can manifest as a pancreatic disorder or another disorder of presumed autoimmune origin. Type 2 disease is typically characterized by absent IgG-4–positive cells. As patients often present with acute pancreatitis, obstructive jaundice, or pancreatic mass, it is imperative to exclude malignancy, a more common diagnosis. AIP may respond to corticosteroids, and has a strong association with other immune-mediated diseases. Recent literature suggests the benefit of immune-modulating therapy, including rituximab, although no consensus exists. This review covers the essentials of diagnosis, but focuses primarily on management of AIP.