This paper illustrates the previously unreported sonographic changes in the biliary tract in metachromatic leukodystrophy (MLD). Gallbladder wall thickening due to sulfatide deposition, intraluminal globules of sulfatide or papillomatosis may cause symptoms referrable to the biliary tract in these patients. A series of patients whom we have studied is briefly alluded to in order to bring to the attention of the radiologist MLD as a rare cause of biliary disease in children.