To determine survival of infants with major congenital anomalies (CA) and assess the effect of co-existing anomalies and gestational age.All liveborn infants with major CA born in New South Wales (NSW), Australia, 2004-2009 were identified from the NSW Register of Congenital Conditions. Deaths were identified via record linkage to death registrations and five-year survival was estimated using Kaplan-Meier methods.There were 8521 liveborn infants with CA of whom 617 (7.2%) died within the first five years of life. Half of deaths occurred in the first week of life. The overall five-year survival rate was 92.8% (95%CI: 92.2-93.3) and 83.2% (95%CI: 79.0-87.4) for syndromes, 83.4% (95%CI: 80.9-85.9) for multiple, 85.1% (95%CI: 82.6-87.5) for chromosomal, 95.3% (95%CI: 94.8-95.8) for isolated and 96.2% (95%CI: 94.3-98.1) for non-Q chapter anomalies. Five-year survival for chromosomal, syndromes and sub-groups was higher for isolated compared with multiple anomalies ranging from 77.5% to 98.9% and 68.6% to 89.5%, respectively. Survival was lower for preterm (79.4%; 95%CI: 77.5-81.4) than for term infants (95.8%; 95%CI: 95.3-96.3).Nine in ten infants with major CA survive up to five years, although there is variability in survival across CA groups. Survival of infants with major congenital anomalies has improved in recent years.