Primary non-Hodgkin's lymphoma of the extrahepatic bile duct presenting as obstructive jaundice is an extremely rare disease. At this writing, a review of the medical literature disclosed 17 reported cases of primary non-Hodgkin's lymphoma arising from the extrahepatic bile duct. We, herein, report an additional case of obstructive jaundice caused by primary non-Hodgkin's lymphoma of the common bile duct, in a 21-year-old woman. Our patient showed clinical evidence of obstructive jaundice, and endoscopic retrograde cholangiopancreatography and abdominal magnetic resonance imaging demonstrated a long strictured segment of the common bile duct with proximal bile duct dilatation. These clinical and radiological findings resembled those of cholangiocarcinoma. Resection of the common bile duct tumor, cholecystectomy, lymph node dissection, and Roux-en-Y hepaticojejunostomy were carried out. Histology and immunohistochemistry of the resected specimen confirmed a diffuse large B-cell-type malignant lymphoma involving the common bile duct. She received four courses of combination chemotherapy, including cyclophosphamide, doxorubicin, vincristine, and prednisone (CHOP), and 3060 cGy external irradiation. She has been well, without evidence of tumor recurrence, 17 months after the surgery. In summary, first, primary non-Hodgkin's lymphoma of the extrahepatic bile duct, despite its rarity, should be considered in the differential diagnosis of causes of obstructive jaundice. Second, an accurate histopathologic diagnosis and surgical resection, if feasible, combined with chemotherapy with or without radiotherapy may be the approach to offer a chance for cure.