Autosomal recessive polycystic kidney disease （ARPKD） is a rare and severe monogenic hereditary disorder characterized by renal collecting duct cyst formation with varying degrees of gallbladder hypoplasia， biliary duct dilatation， and hepatic fibrosis. ARPKD often occurs in the perinatal period or during infancy， and most patients die during the perinatal period or infancy. Therefore， it is of great importance to conduct ARPKD carrier screening and prevent the birth of children with ARPKD. However， there is still a lack of sufficient genetic counseling capacity for ARPKD in China. Based on ARPKD-related studies， guidelines， and consensus statements in China and globally， this consensus systematically summarizes the clinical， medical， and genetic knowledge of ARPKD and elaborates on screening methods， target populations， screening processes， and pre- and post-screening counseling to address the current issues in ARPKD carrier screening， in order to standardize the application of ARPKD carrier screening and better facilitate clinical practice. ［Journal of International Neurology and Neurosurgery, 2024, 51(5): 36-42］