A 61-year-old Japanese male was seen at an outside hospital with abdominal pain and distention. An intraabdominal mass was found on abdominal computed tomography (CT), and he was referred for further evaluation. He was afebrile, with mild tenderness in the upper abdomen and massive distention. Routine blood work showed severe inflammation and anemia. Repeat CT showed a large solitary heterogeneous mass, 25 cm in diameter, in the left upper quadrant with evidence of intratumoral bleeding and irregular enhancement peripherally. There was no clear distinction between the mass and the peripheral organs. Surgery was performed with persistent anemia and exacerbation of abdominal pain. Lateral segmentectomy, total gastrectomy, cholecystectomy and partial resection of the diaphragm and parietal peritoneum were performed. The final diagnosis was cholangiocarcinoma with sarcomatous changes. On the 16th postoperative day a CT scan revealed recurrent tumor. The patient died on the 34th postoperative day from rapid tumor progression.