Biliary disease occurs in a subset of AIDS patients with CD4 counts of less than 100 per mm3. These patients present with right upper quadrant and epigastric pain, cholestasis, and usually abnormal findings on imaging. In 75% of patients, an associated opportunistic infection can be identified. In patients with biliary disease, pain is often relieved following endoscopic sphincterotomy, whereas cholecystectomy provides pain relief in patients with acalculous cholecystitis.