OBJECTIVE Perinatal identification of children with congenital urinary obstruction has challenged our understanding of the pathophysiology and clinical treatment of many children with hydronephrosis. METHODS A critical review of recent reports relating to congenital urinary obstruction in children was performed in an attempt to integrate clinical and experimental information. RESULTS Several themes emerged from the many reports relating to congenital urinary obstruction that have not been previously emphasized. Congenital obstruction begins and evolves in a developing fetal kidney, indicating the importance of the effect of obstruction on renal growth and development, which is distinct from the postnatal renal response to obstruction. Variation in the ability of the developing kidney to compensate for obstruction may be an important factor in explaining variability in clinical and experimental reports. Clinical data show an unpredictable outcome of congenital hydronephrosis. Nonoperative management of hydronephrosis is supported by empirical evidence yet raises many questions regarding the certainty of outcome and the risks involved. It imposes on the practitioner a significant clinical obligation. The ability to differentiate between clinically significant and insignificant obstruction is the current challenge to be fulfilled by integrated clinical and experimental investigation. CONCLUSIONS The unique features of congenital urinary obstruction separate it from better understood acquired postnatal obstruction. Understanding the effects of obstruction on the developing kidney prenatally and postnatally is critical. A definition is proposed for congenital urinary obstruction in children, that is a condition of impaired urinary drainage which, if uncorrected, will limit the ultimate functional potential of a developing kidney.