We report a case of a 62-year-old woman, HIV positive, with a 3-week history of jaundice and elevated cholestatic enzymes. Imaging studies displayed intrahepatic biliary dilatation and a central liver lesion. Pathology described lesions of active cholangitis, lymphoplasmacytic infiltration and fibrosis, suggesting a hepatic inflammatory pseudotumour (IPT) IgG4 related. IgG4-related lymphoplasmacytic form of IPT belongs to IgG4-related diseases. We discuss the importance to include IgG4-related hepatic IPT as part of the differential diagnosis of any liver lesion, highlighting potential imaging clues that may help in establishing the correct diagnosis.