In this study we present a rare case of cholangiocarcinoma with metastasis to the thoracic spine. A 63-year-old female with peripheral cholangiocarcinoma associated with hepatolithiasis presented low back pain as a result of a thoracic spine (T12) compression fracture. Spine surgery and pathological examination revealed a metastatic mucinous adenocarcinoma of unknown origin. Two weeks after the spine surgery, a nontender palpable abdominal mass was found at the epigastric area. Abdominal sonography (US), abdominal computed tomography (CT), and magnetic resonance cholangiopancreaticogram (MRCP) revealed a hepatic tumor, hepatolithiasis, and gallbladder empyema. The patient underwent hepatic resection, cholecystectomy, and choledocholithotomy with T-tube stent. Pathological examination with immunohistochemical stain revealed hepatolithiasis concurrent with mucinous cholangiocarcinoma. Postoperative course was uneventful and no recurrence was noted during a 1-year follow-up period.