A 7-month-old girl with congenital nephrotic syndrome (CNS) of the Finnish type was referred to our group for surgical evaluation and recommendation of a treatment plan. A Medline literature search was conducted using data bases from 1980 to 1996, on the topics of "congenital nephrotic syndrome" and "surgery." Although there were several articles on CNS in the nephrology and pediatric journals, we found none in the urologic literature. Although a few medical centers advocate a less invasive unilateral nephrectomy or percutaneous renal ablation, most institutions still recommend bilateral nephrectomy to manage patients with CNS in whom aggressive medical management fails. Bilateral nephrectomy followed by dialysis and transplantation should remain the standard of care in 1997 for children with severe CNS.