Congenital and inherited kidney diseases are the major causes of childhood uremia, and important causes of adult uremia, while there is a wide variety of rare kidney diseases, most of which are related to inheritance. Environmental factors, genetic factors, and epigenetic factors are important reasons to cause congenital-inherited-rare kidney diseases. With the progress of gene sequencing and bioinformatics analysis technology, many congenital-inherited-rare kidney diseases that were very difficult to diagnose in the past, have gradually been able to be precisely diagnosed by means of clinical, imaging, molecular pathological, and gene detection methods. Progression of these diseases can be delayed through early health management, symptomatic supporting treatment, molecular-mechanism-targeted therapy, gene or stem cell therapy and traditional chinese medicine. Some single-gene genetic diseases, such as autosomal dominant polycystic kidney disease and Alport syndrome, may be prevented from their occurrence through the in vitro fertilization and embryo selection techniques. Therefore, attention should be paid to the basic and clinical research of congenital-inherited-rare kidney diseases, striving for precise diagnosis and effective treatment, blocking disease inheritance, protecting renal function, and improving quality of life of patients.