A 20-year-old male presented with hepatomegaly, jaundice, and itching. Investigation revealed elevated liver enzyme levels, suggesting direct hyperbilirubinemia. Magnetic resonance imaging revealed a stable orientation of the right hemithorax, a relatively small and rotated right hepatic lobe, and a left-rotated intra-abdominal hepatic lobe, with progressive enhancement related to the foci of the hepatic fibrosis. There was an intrahepatic biliary dilatation, reaching 0.8 cm, that was due to biliary compression. There was no evidence of biliary irregularity to suggest a classic primary sclerosing cholangitis pattern. Percutaneous transhepatic biliary drainage and a cholangiogram confirmed the dilation of the left and right bile ducts with smooth, normal drainage via the common bile duct. The symptom management strategy took the form of surgical liver transplantation. The patient demonstrated improvement following transplantation, and his total bilirubin level returned to normal within months. The patient was then discharged in a stable status, and successive follow-up appointments were scheduled every three months to monitor for disease progression or complications.