This report describes 3 cases of juvenile dermatomyositis (juvenile DM) complicated by cholestasis. All 3 patients had typical features of juvenile DM, and all developed a cholestatic syndrome within the initial months of their disease. Liver biopsy revealed mixed (cytoplasmic and ductal) cholestasis with no abnormalities in the intrahepatic ducts in all 3 cases. Cholestasis improved or was completely reversible upon treatment with prednisone. In the 2 patients who could be followed up long term, no sequelae remained. The possible role of inflammation in the pathogenesis of cholestasis in juvenile DM is discussed.