Abstract
Autoimmune pancreatitis (AIP) is a rare form of pancreatitis. This case report focuses on type 1 autoimmune pancreatitis (AIP-1), an immunoglobulin G4 (IgG4)-related disease. It is characterized by dense infiltration of lymphocytes and plasma cells, primarily in a periductal distribution. We report a case of a 48-year-old man diagnosed with AIP-1 who subsequently developed eccrine chromhidrosis and bilioptysis during the peak of bilirubin level. This case highlights the diagnostic process followed to identify this challenging condition and demonstrates the patient's response to the mainstay treatment. Moreover, it discusses the rare symptoms that can manifest with marked hyperbilirubinemia.