Undifferentiated spindle and giant cell carcinoma is an extremely rare malignant neoplasm arising in the extrahepatic bile duct. We herein present the case of a 67-year-old male who developed an undifferentiated spindle and giant cell carcinoma of the proximal bile duct. A nodular infiltrating tumor was located at the proximal bile duct, resulting in obstructive jaundice. Histologically, the tumor was composed of mainly spindle-shaped and giant cells and showed positive immunoreactivity for both cytokeratin and vimentin. Adjuvant chemotherapy was administered following extrahepatic bile duct resection, and he has been doing well for 16 months since the surgical treatment. The literature on this rare malignancy is also reviewed and discussed.