This study aimed to investigate the survival of infants born with spina bifida between 1979 and 1994 from the population-based Metropolitan Atlanta Congenital Defects Program (MACDP) and to identify clinical and demographic factors associated with survival. Survival status was obtained from MACDP records and the National Death Index. Survival rates were calculated using the Kaplan–Meier method. Risk factors potentially associated with survival were examined by the log-rank test. We assessed the independent effect of risk factors using the Cox proportional hazards model. Overall, 78.4% of children with spina bifida survived during the study period. Of the 235 infants born with spina bifida, 87.2% survived the first year of life. Survival to age 1 for the 1979–83, 1984–88 and 1989–94 birth cohorts was 82.7%, 88.5% and 91.0% respectively. In multivariable analysis, factors associated with increased mortality were low birthweight (<2500 g) (vs. ≥2500g, relative risk (RR) 2.3 [95% CI 1.1, 4.9]) and high lesions (vs. low lesions, RR 3.4 [95% CI 1.6, 7.1]). This study suggests a continuous improvement in survival among children born with spina bifida in Atlanta. Demographic and clinical factors are associated with length of survival. This information is useful for both clinicians and families who need to plan for the long-term care of these children.