Low phospholipid-associated cholelithiasis (LPAC) is characterized by a low biliary phospholipid concentration with symptomatic and recurring cholelithiasis, and LPAC syndrome is associated with mutations of the adenosine triphosphate-binding cassette, subfamily B, member 4 (ABCB4) gene encoding the hepatobiliary phospholipid translocator multidrug resistance protein 3 [37]. Here, ABCB4 is linked to cholelithiasis.