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Atypical presentation of IgG IgG4 sclerosing cholangitis (ISC) is a rare disease which usually presents in patients in their 60's or above. Patients often present with obstructive jaundice as the first symptom, especially when associated with autoimmune pancreatitis. We report the case of a 20-year-old man who presented with acute abdominal pain, which was subsequently diagnosed as ISC in addition to autoimmune pancreatitis following magnetic resonance cholangiopancreatography (MRCP). The patient was thus treated with steroids.