During the 3-year period, January 1986 through December 1988, 7 of 24 infants evaluated for cholestatic liver disease were found to have choledocholithiasis with or without associated gallbladder disease. Five were born prematurely with associated predisposing factors, especially systemic infection and total parenteral nutrition. The clinical course varied from spontaneous recovery in two patients to ongoing liver disease requiring surgical or endoscopic intervention in five infants. Asymptomatic patients with normal liver function and persistently dilated extrahepatic biliary trees posed the most difficult therapeutic problems. Endoscopic retrograde cholangiography appeared to be an effective and rewarding therapeutic approach.