The establishment of differentiation protocols of kidney organoids from human pluripotent stem cells (hPSCs) provides potentials in applications of kidney organoids in regenerative medicine. Modeling of genetic kidney diseases and investigating new therapeutic option may be an attractive an application. Although much progress still remains to be made in the development of kidney organoids, recent advances in CRISPR-Cas9 genome editing have contributed to the application of kidney organoids in genetic kidney disease. In this session, I will review the recent advances and share our experiences in the applications of kidney organoids in genetic kidney diseases.