Abstract The term ‘congenital nephrotic syndrome’ (CNS) is used to designate nephrotic syndrome (NS) which is present at birth or, by extension, detected within the first three months oflife, whereas ‘infantile nephrotic syndrome’ applies to NS of later onset, up to one year of age. Most have a genetic basis and a poor outcome. The best example of CNS was initially observed and described in Finland by Hallman and Hjelt (1959) and is now called congenital nephrotic syndrome of Finnish type.