Congenital mesoblastic nephroma (CMN) accounts for approximately 5% of paediatric renal tumours with the highest peak of incidence during the first 3 postnatal months. CMN almost always has a favourable prognosis. Therefore, CMN needs to be correctly diagnosed and differentiated, principally histologically, from other pediatric neoplasms. We present a clinicopathologic study of 5 cases of CMN.