Approximately 1.3% of patients with lymphoma develop obstructive jaundice secondary to lymphomatous involvement of the extrahepatic biliary system. This may occur either as an initial or as a late manifestation of disease. Clinically and radiographically the condition may mimic a variety of more common causes of obstructive jaundice. Surgical exploration may be necessary to confirm the diagnosis, but local radiotherapy would appear to be the preferred mode of treatment. Rapid progression to systemic disease occurs in the majority of patients, necessitating multidrug chemotherapy. Control of jaundice by radiotherapy is good, but long-term prognosis is poor. Supervening sepsis and gastrointestinal bleeding caused the majority of deaths, suggesting that adjunctive nutritional support, immunologic enhancement, and stress ulcer prophylaxis may be necessary if survival is to be improved.