BackgroundCholangiocarcinomas are rare tumors, and metastasis to the intramedullary spinal cord is also rare. To the best of our knowledge, this is the first case of simultaneous cholangiocarcinoma and intramedullary spinal cord metastasis to be described in the medical literature.Case presentationA 62-year-old Caucasian male with a cholangiocarcinoma presented pain around his left shoulder without any other symptoms. The results by magnetic resonance imaging and F18 fluorodeoxyglucose positron emission tomography/computer tomography revealed an intramedullary metastasis at the C4 level, with spinal cord compression, and numerous secondary parenchymal brain metastases.ConclusionThis patient was treated successfully with a combination of radiotherapy, corticosteroids, and chemotherapy. He experienced complete relief of the symptoms and showed improvements upon subsequent radiological evaluations.