Congenital nephrotic syndrome is a rare clinical entity defined as massive proteinuria leading to symptoms within the infant's first 3 months of life. Although the association between congenital nephrotic syndrome and cytomegalovirus infection has been identified, association with haemophagocytosis has not been reported in the literature. In this case report we describe concomitant cytomegalovirus infection and haemophagocytosis in a 3-month-old girl with congenital nephrotic syndrome.