Diagnosis of primary sclerosing cholangitis (PSC) is often very difficult and may have a suspicion with altered liver functions. PSC is known to be associated with inflammatory bowel disease. This article presents a case study of a 70 years old male patient who presented with obstructive jaundice with recurrent episode of cholangitis in June 2019 at Bangabandhu Sheikh Mujib Medical University (BSMMU), Dhaka, Bangladesh. Evaluation revealed beaded appearance in MRCP with positive relevant markers which raised suspicion of PSC and it was confirmed by biopsy and histopathology of the affected segment in biliary tree. The importance of early detection of primary sclerosing cholangitis in an effort to decrease the morbidity and mortality from cholangiocarcinoma will also be emphasized and our management according to local protocol and outcome of this patient.
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