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Inspissated bile syndrome (IBS) is a relatively rare condition. Many treatment options are available, including medication, surgery,and surgical interventions, such as insertion of cholecystostomy drain, endoscopic retrograde cholangiopancreatography,internal biliary drainage, and percutaneous transhepatic biliary drainage (PTBD). We herein report the first case of IBS that wassuccessfully treated with PTBD in a two-month-old infant in Korea. PTBD was initiated on postnatal day 72. On postnatal day105, we confirmed complete improvement and successfully removed the catheters. This report suggests that PTBD is a viable andsafe treatment option for obstructive jaundice in very young infants