• The clinical efficacy of percutaneous ethanol sclerotherapy is very high. • Haemorrhagic content should not be a contraindication for percutaneous sclerotherapy. • Dominant cysts on polycystic liver disease should be treated with PES. • Imaging follow-up should not be performed shortly after the procedure.

BackgroundABCB4/MDR3 gene variants are mostly associated with a peculiar form of cholelithiasis in European adults, currently referred to as low phospholipid-associated cholelithiasis (LPAC) syndrome.MethodsLPAC syndrome is a rare genetic disorder, characterised by the following clinical features: biliary symptoms before the age of 40, recurrence of the symptoms after cholecystectomy, and intrahepatic microlithiasis or intrahepatic hyperechogenic foci.ResultsImaging features associated with ABCB4/MDR3 mutations are not specific and correspond to a wide spectrum of biliary abnormalities. The main feature is the presence of intrahepatic lithiasis. Other uncommon presentations have been described, such as uni- or multifocal spindle-shaped dilatations of the intrahepatic bile ducts filled with gallstones, secondary sclerosing cholangitis, biliary cirrhosis, and intrahepatic cholangiocarcinoma.ConclusionThis review focuses on MR features related to ABCB4/MDR3 mutations.Main Messages• LPAC syndrome is characterised by intrahepatic microlithiasis or intrahepatic hyperechogenic foci.• Ultrasound examination is very accurate in detecting intrahepatic stones.• At MR imaging, LPAC syndrome is associated with various presentations.