Objective To summarize the etiology and clinical features of 50 children with stage 5 chronic kidney disease (CKD) treated at a tertiary hospital in western China in the past 10 years and to establish a theoretical basis for the investigation of chronic kidney disease in children. Methods The clinical data of 50 children aged 0-18 years with CKD5 who visited the General Hospital of Ningxia Medical University from January 1, 2010 to June 30, 2021 were retrospectively collected, and their etiological composition and clinical features were analyzed. Results Among the 50 children with CKD stage 5, 31 (62.0 %) were male and 19 (38.0 %) were female. The age of onset was 0.5-18 years old, including 8 cases (16.0 %) aged 0-6 years, 17 cases (34.0 %) 7-12 years old, and 25 cases (50.0 %) aged 13-18 years. At the initial diagnosis, there were 9 cases (18.0 %) with no obvious symptoms, 15 cases (30.0 %) with primarily renal symptoms, and 26 cases (52.0 %) with mainly extrarenal symptoms. The primary cause of the disease was glomerular disease in 26 cases (52%) and congenital renal and urinary tract malformations in 12 cases (24.0 %). Complications observed were anemia in 49 cases (98.0 %), chronic kidney disease-mineral and bone abnormalities (CKD-MBD) included hyperphosphatemia in 49 cases (98.0 %), secondary hyperparathyroidism in 38 cases (76.0 %), hypocalcemia in 33 cases (66.0 %), vitamin D deficiency in 27 cases (54.0 %), water electrolyte acid-base disorders including hyperuricemia in 40 cases (80.0 %), hyperkalemia in 15 cases (30.0 %), hypertension in 28 cases (56.0 %), growth disorder in 18 cases (36.0 %), and refinement of nephropuncture biopsy in 7 cases (14.0 %), exoms sequencing in 5 cases (10.0 %). loss of follow-up in 2 cases (4.0 %), death in 11 cases (22.0 %), long-term hemodialysis in 15 cases (30.0 %), long-term peritoneal dialysis in 13 cases (26.0 %), and kidney transplantation in 11 cases (22.0 %). Conclusions The main cause of children with CKD stage 5 in this region is glomerular disease; the incidence rate is higher in males than females, and higher in older than younger children; the first symptom is more common with extra-renal manifestations, and the most common complications are anemia and CKD-MBD; the prognosis of children who have completed renal transplantation is better, but the vast majority of them opt for dialysis treatment, which is predominantly hemodialysis; and the main causes of death are severe infections and diseases of cardiovascular system.