Sixty-six children (35 boys and 31 girls) aged 14 years or younger, with chronic renal failure (CRF), were reviewed in the department of pediatrics at The King Abdul Aziz University Hospital (KAUH), Jeddah, over a four-year period from September 2000 until July 2004. Fifty-nine percent (39 patients) were Saudi Nationals while the remaining were from other nationalities. Forty-two percent lived outside Jeddah in other cities of the western or the southern provinces. Their mean glomerular filtration rate (GFR) was 15.3 +/- 11.1 ml/minute/1.73m(2); 50patients (76%) had severe CRF with GFR of < 25 ml/minute/1.73m(2), of whom 34 (52%) were in end-stage renal failure (ESRF), with GFR < 10 ml/minute/1.73m(2). The mean age at first presentation was 4.5 +/- 4.3 years, while the mean age at referral to a pediatric nephrologist was 6.6 +/- 4.4 years. Congenital abnormalities of the renal system were the major cause of CRF (33 patients, 50%) followed by neurogenic bladder (19.6%), either idiopathic (6%) or associated with neural tube defects (13.6%). Hereditary conditions were the cause in 12% and glomerular disease in 13.6%. Fourteen children (21.2%) received peritoneal dialysis, seven (10.6%) received hemodialysis, two (3%) were transplanted abroad and 12 patients (18%) died. Our study, which is the first from Jeddah on the epidemiology of CRF in children, shows that the profile is similar to other parts of the KSA with a predominance of congenital causes. There was a considerable delay in referring children with CRF patients to a pediatric nephrologist resulting in delay in the management of preventable causes such as neurogenic bladder associated with neural tube defects.