Although hepatobiliary involvement is common in the acquired immunodeficiency syndrome, it infrequently leads to biliary tract abnormalities.We describe a 39-year-old man with human immunodeficiency virus infection and no previous acquired immunodefiency syndrome-defining illnesses, who presented with malaise, right upper quandrant pain, lymphadenopathy and cholestasis. An endoscopic retrograde cholangiopancreatography demonstrated sclerosing cholangitis due to disseminated B-cell non-Hodgkin's lymphoma. Following chemotherapy, his symptoms and signs rapidly improved, so that 1 month later his endoscopic retrograde cholangiopancreatography has returned entirely to normal.