Abstract:Hereditary spherocytosis (HS) is a hereditary hemolytic disease with a global distribution, which often presents clinically with anemia, jaundice and splenomegaly. The diagnosis and treatment of HS has progressed significantly in recent years. Cholelithiasis is the most common complication, seriously affecting the health of HS patients. It has been demonstrated that patients with HS associated with cholestasis have disturbed bile acid metabolism, which affects the composition and abundance of gut microbiota. This article reviewed the role and mechanism of bile acid metabolism-gut microbiota interactions in regulating HS associated with cholestasis, with the aim of providing novel treatment strategies for HS associated with cholestasis.