Finnish congenital nephrosis is a very rare autosomal recessive form of nephrotic syndrome. It is seen more commonly in families of Finnish descent, with an incidence of 1:10,000 births, but can affect every race, with a considerably lower frequency. It is a distinct clinical entity involving massive proteinuria, prematurity, large placenta, hypoproteinemia and marked edema. The typical histological findings of Finnish congenital nephrosis kidneys are dilated proximal tubules, mesangial hypercellularity, and glomerular fibrosis and sclerosis. This progressive disease leads to death in the first two years of life; the only curative therapy is bilateral nephrectomy followed by renal transplantation.