Background: VACTERL association is defined in the presence of at least four of the following abnormalities: vertebral anomalies, anal atresia, cardiac malformations, tracheoesophageal fistula, renal anomalies, and limb anomalies. The aim of this study was to determine the renal status in our group of VACTERL patients. Patients and methods: Diagnosis was established on the basis of abovementioned criteria. Following imaging studies were performed: ultrasound scan, Tc99mDMSA scan, Tc99mDTPA scan, IVP and VCUG as indicated. Proteinuria was assessed in 24 hour urine samples or as protein/creatinine ratio in random samples. GFR was calculated according to Schwartz formula. Resu lts: There were four children (3 males, one female) who fulfilled at least four criteria for VACTERL and all had renal abnormalities. The female had unilateral multicystic kidney dysplasia with spontaneous resolution of the cysts. One male had bilateral ureterohydronephrosis with afunctional and dysplastic right kidney with ectopic insertion of the right ureter. The second male had non-obstructive duplex anomaly. The third male had single, ectopic and dysplastic kidney and neurogenic bladder and recurrent episodes of pyelonephritis. The renal function remained normal in 3 children, the boy with single dysplastic kidney and neurogtenic bladder progressed to renal insufficiency. Conclusion: In children with VACTERL association renal status and function should be monitored periodically, particularly in those with single and dysplastic kidneys. Although these children survive neonatal period, long term prognosis is dependent on the severity of renal abnormalities.