A female patient, aged 60, was hospitalized mainly due to recurrent epigastric pain for 30 years, which was accompanied by chills and high fever for half a year. The patient did not have nausea, vomiting, black stool, hematemesis, or obvious jaundice. Intermittent symptoms of slight upper abdominal pain occurred after surgery without other discomfort. She described a slight loss of appetite and normal bowel and bladder function. The patient did not receive any medications prior to admission. She underwent open cholecystectomy for gallstones more than 30 years ago, and the surgical records were not found. Adhesiolysis was performed 20 years ago because of adhesive intestinal obstruction. She denied alcohol assumption and smoke, and she took no medications. She denied any other personal medical history or a family history. The patient's body mass index (BMI) was 18.5. On admission, physical examination revealed mild jaundice of bilateral sclera, surgical incision scar in the abdomen, tenderness in the right upper abdomen, no rebound pain or muscle tension, positive tapping pain in the hepatic region, and no abnormal bowel sounds. The laboratory test results are shown in Table. The results of abdominal enhanced CT examination showed that there was no gallbladder, the edge of the liver was not smooth, intrahepatic bile duct stones were accompanied by bile duct dilatation, the morphology of the right lobe of the liver was abnormal, the left and right hepatic veins were narrowed, and the middle hepatic vein was not shown. The size of the spleen and pancreas was normal. Magnetic resonance cholangiopancreatography (MRCP) showed intrahepatic bile duct dilatation with stones, no gallbladder was shown, the common bile duct and hepatic duct were not clearly visualized, the shape of the right lobe of the liver was abnormal with stasis of hepatic lymph nodes, and the pancreatic duct was well developed without dilatation or stenosis.