Abstract: Congenital cytomegalovirus (CMV) infections remain the leading viral cause of congenital malformations in the developed world. Despite advances in our knowledge, the epidemiology and natural history of congenital CMV infection are still poorly understood, particularly in Australia. Congenital CMV causes illness ranging from no clinical disease (asymptomatic, but infected) through to prematurity, encephalitis, deafness and haematological disorders and death. Perinatal CMV acquisition usually results in less severe illness including asymptomatic infection, acute infection with hepatitis, fever, and pneumonitis. CMV infects only humans, and in vitro and in vivo models for intrauterine infection are required in order to test new treatments, and better describe the pathogenesis of congenital CMV. Using new knowledge of the epidemiology and natural history of CMV, treatment regimens during late pregnancy are currently undergoing clinical trial although no definitive recommendations are available.