Human immunodeficiency virus (HIV) infection has been associated with a number of hepatic and biliary tract disorders. Case reports, series of liver biopsies, and postmortem studies that examined the hepatobiliary system were retrieved with a MEDLARS search and form the basis of this review. The liver and biliary tract are frequently involved with opportunistic infections (most commonly mycobacteria and cytomegalovirus) and neoplasms (mainly Kaposi's sarcoma) in patients with HIV infection. The patients are often asymptomatic but may have elevated levels of serum liver enzymes. These abnormalities are nonspecific. Sulfa drugs, pentamidine, and ketoconazole are the medications used in HIV-related infections that are most likely to result in abnormalities on liver tests. Acalculous cholecystitis and sclerosing cholangitis also occur in HIV infection. Cytomegalovirus and Cryptosporidium are the organisms most commonly associated with these conditions. Imaging studies of the liver may detect parenchymal abnormalities and guide liver biopsy. The role of this procedure in the diagnosis of opportunistic infections and neoplasms is controversial because these lesions are generally disseminated at the time liver abnormalities are evident. A liver biopsy is best used when other less invasive procedures have failed to provide a diagnosis. Endoscopic retrograde cholangiopancreatography is a useful diagnostic procedure with therapeutic potential in patients with abdominal pain, fever, or an elevated serum alkaline phosphatase level.