SUMMARY
Congenital abnormalities of the kidney and urinary tract (CAKUT) are one of the leading congenital defects to be identified on prenatal ultrasound. CAKUT represents a broad spectrum of abnormalities, from transient hydronephrosis to severe bilateral renal agenesis. CAKUT is a major contributor to chronic and end stage kidney disease (CKD/ESKD) in children. Both genetic abnormalities and the fetal environment contribute to CAKUT. Monogenic gene mutations identified in human CAKUT have advanced our understanding of molecular mechanisms of renal development. Low nephron number and solitary kidneys are associated with increased risk of adult onset CKD and ESKD. Premature and low birth weight infants represent a high risk population for low nephron number. Additional research is needed to identify modifiable factors to enhance nephron development in premature infants and biomarkers and appropriate follow-up of premature and low birth weight infants into adulthood.