We present a fatal case of neonatal invasive group A streptococcal disease and review of the literature. Twenty-four cases were early onset disease and were associated with concurrent maternal infection, respiratory distress, pneumonia, toxic shock-like syndrome and serotype M1. Fifteen cases were late onset disease associated with soft tissue infections and meningitis. Maternal carriage was identified as an important factor in neonatal group A streptococcal disease.