Autoimmune pancreatitis is the pancreatic manifestation of a systemic disorder that affects various organs, including the bile duct, retroperitoneum, kidney, and parotid and lacrimal glands. It represents a recently described subset of chronic pancreatitis that is immune mediated and has unique histologic, morphologic, and clinical characteristics. A hallmark of the disease is its rapid response to corticosteroid treatment. Although still a rare disease, autoimmune pancreatitis is increasingly becoming recognized clinically, leading to evolution in the understanding of its prognosis, clinical characteristics, and treatment.