Here we summarize the discussions and provide an up-dated set of diagnostic, surveillance, and management rec-ommendations for optimizing the pediatric care of patients with ARPKD. Specialist care of ARPKD-related complica-tions, including dialysis, transplantation, and management of severe portal hypertension (HTN), will be addressed in a subsequent report. Given the paucity of information regarding targeted therapies in ARPKD, this topic was not addressed in this conference.