Autoimmune pancreatitis (AIP) is a rare form of chronic pancreatitis characterized by inflammation and fibrosis, often misdiagnosed due to its similarity with other pancreatic diseases. AIP is classified into two main types: Type 1 AIP (AIP-1) is associated with elevated serum immunoglobulin G4 (IgG4) levels and systemic manifestations, while Type 2 AIP (AIP-2) is a more localized form often coexisting with inflammatory bowel diseases (IBDs). A third type, Type 3 AIP, refers to immunotherapy-triggered AIP. Clinical manifestations include painful jaundice and weight loss. Diagnosis involves a combination of clinical, laboratory, radiological, and histological findings. Steroid therapy is the primary treatment, with additional immunosuppressive agents used in severe cases. The high relapse rate in AIP-1 necessitates long-term maintenance therapy. The article reviews the current knowledge of AIP, including epidemiology, etiology, clinical presentation, diagnosis, and treatment options, highlighting the need for increased awareness and knowledge of this complex disease.Autoimmune pancreatitis (AIP) is a rare form of chronic pancreatitis characterized by inflammation and fibrosis, often misdiagnosed due to its similarity with other pancreatic diseases. AIP is classified into two main types: Type 1 AIP (AIP-1) is associated with elevated serum immunoglobulin G4 (IgG4) levels and systemic manifestations, while Type 2 AIP (AIP-2) is a more localized form often coexisting with inflammatory bowel diseases (IBDs). A third type, Type 3 AIP, refers to immunotherapy-triggered AIP. Clinical manifestations include painful jaundice and weight loss. Diagnosis involves a combination of clinical, laboratory, radiological, and histological findings. Steroid therapy is the primary treatment, with additional immunosuppressive agents used in severe cases. The high relapse rate in AIP-1 necessitates long-term maintenance therapy. The article reviews the current knowledge of AIP, including epidemiology, etiology, clinical presentation, diagnosis, and treatment options, highlighting the need for increased awareness and knowledge of this complex disease.