Kidneys as well as urinary tracts are the major excretory organs of the human body and removes waste products of protein metabolism. They also remove surplus water and salts from the blood. Kidneys as well as urinary tracts also important for survival as they are crucial for preserving the fluid-electrolyte balance of the body tissue fluids. The inaccuracies in the embryogenesis of the outflow tracts along with the kidneys are vulnerable to the environmental endangerments and disturb the development throughout gestation. Mutations in the patient with CAKUT are responsible for the chronic renal diseases in the 1st three decades of life. Fetus dose not survive in severe cases of CAKUT as a result of non-formation of kidneys and outflow tract, but in less severe cases there are more chances to survive and may identified in adulthood. Though the critical cases of CAKUT are infrequent, marginal nephron number is considerably frequent disorder exerting its effect on renal functions with increasing age. Congenital anomalies of kidney and urinary tract have a wide range of abnormalities inclusive of fatal agenesis of both the kidneys and asymptomatic ectopic kidneys. Hence patient with the solitary kidney should follow-up regularly for better and close monitoring. The clinical presentation of CAKUT along with its long term outcomes and epidemiology is covered in this review. With a comprehensive knowledge of the roots of CAKUT and marginal nephron numbers, identification of preventive treatment and establishment of clinical guidelines for the patients can be begun. Comprehension of relevant endangerments and changes in the epidemiological trends especially modified maternal factors and delivery services is vital for prompt detection and prediction of prognosis in the course of pregnancy due to subsequent morbidity and mortality as a result of the presence of CAKUT.
