We evaluated all fatal neonatal sepsis and pneumonia cases occurring in Alaska during 1992-2000. Risk factors were evaluated using a database of all births occurring during the study period. Of 32 cases, group B streptococcus (GBS) was isolated from 21% (all 7 days of age), non-GBS Gram-positive bacteria from 50% (53% <7 days of age), and Gram-negative infections from 38% (58% <7 days of age). Infants born at <37 weeks gestation accounted for 72% of cases and had an increased risk of GBS [rate ratio (RR) 9.1, 95% confidence interval (CI) 2.0-41] and non-GBS (RR 40, 95% CI 16-101) disease. Neonatal sepsis mortality has become an outcome concentrated among pre-term infants. Aetiologies include GBS during the early neonatal period, Candida spp. during the late neonatal period, and other bacteria during both periods.