INTRODUCTION: We evaluated outcomes in children with chronic kidney disease stage 5 (CKD 5) treated in the first pediatric dialysis unit in Poland during 1973-2012. MATERIAL AND METHODS: The retrospective analysis included 208 children with CKD 5 undergoing renal replacement therapy (RRT), stratified into four decades of treatment: 1973-1982, 1983-1992, 1993-2002, and 2003-2012. RESULTS: The most common causes of CKD 5 included glomerulonephritis in 27.4% and pyelonephritis secondary to urinary tract anomalies in 25.5% of children. Among 208 children, 172 (82.7%) survived and 17.3% died. Kidney transplantation (KTx) was performed in 47.6% of children, including pre-emptive KTx in 1.92% of children. Chronic dialysis was continued in 34.1% of children, and RRT was withdrawn in 1%. The overall mortality rate was 6.2 per 100 patient-years, and 3-year survival was 83.9%. The highest mortality rate of 23.4 per 100 patient-years was observed among children in whom RRT was initiated in 1973-1982, with subsequent reduction of the mortality rate to 4.5 and 2.1 per 100 patient-years in 1993-2002 and 1983-1992 respectively. No deaths were noted after 2002. Cardiovascular problems were the most common cause of death, found in 36.1% of patients (p < 0.01). Identified risk factors for mortality included young age, low residual diuresis, anemia at the time of RRT initiation, and hypertriglyceridemia and hypoalbuminemia during RRT. CONCLUSIONS: In years 1973-2012 significant improvement in prognosis among children with CKD 5 was achieved. Identified predictors of mortality included young age at initiation of RRT, low residual diuresis, anemia and hypertriglyceridemia.