This report describes one case of primary non-Hodgkin lymphoma of the extrahepatic biliary tree. The main symptom was obstructive jaundice. Cholangiography demonstrated stricture of the bile duct which resembled the appearance of cholangiocarcinoma. The surgical approach allowed complete ressection. The histopathological analyses showed a centrocitic-centroblastic follicular non-Hodgkin lymphoma. She underwent chemotherapy, developed severe bone marrow hypoplasia, but 48 months after surgery, the patient is doing well.