Autoimmune pancreatitis (AIP) is a rare, chronic autoimmune disease of the pancreas caused by an aberrant immune response leading to inflammation and fibrosis. It is classified into three types: AIP-1 (lymphoplasmacytic sclerosing pancreatitis), AIP-2 (idiopathic ductal centric pancreatitis), and AIP-3 (immunotherapy-triggered AIP). AIP-1 is associated with elevated serum IgG4 levels and systemic manifestations, while AIP-2 is more localized and often coexists with inflammatory bowel disease. AIP-3 is a rare form that occurs in patients with advanced malignancies and is triggered by immune checkpoint inhibitors. The clinical presentation of AIP includes painless jaundice, weight loss, and pancreatic dysfunction. Diagnosis relies on a combination of clinical, laboratory, radiological, and histological findings. The mainstay of treatment for AIP is steroid therapy, which is effective in most cases. Severe cases may require additional immunosuppressive agents. This review summarizes current knowledge of AIP, including its epidemiology, etiology, clinical presentation, diagnosis, and treatment options. It also addresses challenges and controversies in diagnosing and treating AIP, such as distinguishing it from pancreatic cancer and managing long-term treatment. Scientific efforts are focusing on target therapies. AIP-1 is more common in Asia than in the United States and Europe, while AIP-2 is more prevalent in Western countries. AIP-3 is associated with immune checkpoint inhibitors and is not characterized by pathognomonic histopathologic lesions. The diagnosis of AIP requires histological evaluation, and the presence of GELs is a key feature of AIP-2. Steroids are the first-line therapy for both AIP-1 and AIP-2, and rituximab is a good alternative for AIP-1. Given the high relapse rate, long-term maintenance therapy is recommended. Target therapies are being explored for AIP. The role of immunosuppressants and biologics in the treatment of AIP is also discussed. The review highlights the need for increased awareness and knowledge of this complex disease.Autoimmune pancreatitis (AIP) is a rare, chronic autoimmune disease of the pancreas caused by an aberrant immune response leading to inflammation and fibrosis. It is classified into three types: AIP-1 (lymphoplasmacytic sclerosing pancreatitis), AIP-2 (idiopathic ductal centric pancreatitis), and AIP-3 (immunotherapy-triggered AIP). AIP-1 is associated with elevated serum IgG4 levels and systemic manifestations, while AIP-2 is more localized and often coexists with inflammatory bowel disease. AIP-3 is a rare form that occurs in patients with advanced malignancies and is triggered by immune checkpoint inhibitors. The clinical presentation of AIP includes painless jaundice, weight loss, and pancreatic dysfunction. Diagnosis relies on a combination of clinical, laboratory, radiological, and histological findings. The mainstay of treatment for AIP is steroid therapy, which is effective in most cases. Severe cases may require additional immunosuppressive agents. This review summarizes current knowledge of AIP, including its epidemiology, etiology, clinical presentation, diagnosis, and treatment options. It also addresses challenges and controversies in diagnosing and treating AIP, such as distinguishing it from pancreatic cancer and managing long-term treatment. Scientific efforts are focusing on target therapies. AIP-1 is more common in Asia than in the United States and Europe, while AIP-2 is more prevalent in Western countries. AIP-3 is associated with immune checkpoint inhibitors and is not characterized by pathognomonic histopathologic lesions. The diagnosis of AIP requires histological evaluation, and the presence of GELs is a key feature of AIP-2. Steroids are the first-line therapy for both AIP-1 and AIP-2, and rituximab is a good alternative for AIP-1. Given the high relapse rate, long-term maintenance therapy is recommended. Target therapies are being explored for AIP. The role of immunosuppressants and biologics in the treatment of AIP is also discussed. The review highlights the need for increased awareness and knowledge of this complex disease.