Lymphoma is a rare cause of biliary obstruction and, on cholangiography, may mimic other causes of obstructive jaundice. The optimum treatment for these patients is unclear. The aim of this study is to evaluate the incidence, clinical and imaging findings, management, and outcome of biliary obstruction caused by lymphoma. Our database was searched retrospectively for patients with biliary obstruction due to lymphoma between 1999 and 2005. Biliary obstruction secondary to lymphoma was found in 7 (0.6%) of 1123 patients with malignant biliary obstruction. One patient had benign biliary obstruction related to lymphoma. Of the eight patients (five male, three female; mean age, 34.50 +/- 17.93 years), four had Hodgkin's disease and four had non-Hodgkin's lymphoma. Biliary obstruction occurred as part of the initial or early presentation of lymphoma in two patients. The most common cause of obstruction was compression of the biliary tract by enlarged lymph nodes (six patients). Cholangiographic appearances were diverse: narrowing of the common bile duct (six patients), splayed and narrowed common bile duct (one patient), and multiple strictures and dilatations of the intrahepatic bile ducts (one patient). Biliary drainage was performed in all patients including endoscopic stent placement in six patients, nasobiliary drainage in one, and choledochoduodenostomy in one. Hyperbilirubinemia resolved in all but one of the patients with a stent; however, none could be maintained in a stent-free condition. Five patients died within 1 year after onset of jaundice. One of the surviving patients developed a late benign stricture at the site of the earlier lymphoma. We conclude that lymphoma should be considered in the differential diagnosis of obstructive jaundice, particularly in younger patients. We suggest that biliary drainage by the endoscopic or percutaneous route is necessary for the treatment of these patients. Late benign strictures may develop. Biliary obstruction is a sign of poor prognosis in lymphoma.