Squamous cell carcinoma of the extrahepatic bile duct is very rare. Only limited information isavailable concerning its pathogenesis and prognosis, although it is reported to be associatedwith choledocolithiasis, recurrent pyogenic cholangitis, clonorchiasis, choledochal cyst formation,and primary sclerosing cholangitis. Therefore, we present a case of squamous cell carcinoma ofthe distal common bile duct. A 64-year-old male patient presented with abdominal pain andobstructive jaundice. Computed tomography and endoscopic retrograde cholangiographyshowed thickening of the wall and a segmental stricture of the distal common bile duct, and thebiopsy confirmed poorly differentiated carcinoma with marked squamous differentiation. Thepatient underwent a pylorus-preserving pancreaticoduodencetomy. Histological examinationrevealed that the tumor invaded the pancreas and metastasized to regional lymph nodes(pT3N1M0). The patient developed multiple liver metastases 30 days later and died of rapidlyprogressive metastatic disease to the liver 5 months following resection. Squamous cellcarcinoma of the distal bile duct with lymph node metastasis had a very poor prognosis, eventhough curative resection was performed.