HIV cholangiopathy is a disease of advanced-stage AIDS that presents with biliary symptoms and anicteric cholestasis. An abnormal ultrasound examination in a patient with low CD4 count is evaluated by endoscopic retrograde cholangiopancreatography, which demonstrates the characteristic cholangiographic abnormalities. Besides being the gold standard for diagnosis, it offers therapeutic intervention and possible pain relief in the presence of papillary stenosis. An infectious pathogen is identifiable in a majority of patients, suggesting infection-related damage to the biliary tree. Anti-infective therapy, however, usually is ineffective, and prognosis is related to the underlying stage of AIDS.