BACKGROUND: The objective is to study racial differences in infant mortality attributable to birth defects (IMBD) in the United States. METHODS: We analyzed 1989–1991 and 1995–2002 linked birth/death files for trends and racial differences in IMBD by selected categories of birth defects for infants of non-Hispanic white, non-Hispanic black, and Hispanic mothers. RESULTS: In 1989–2002, the IMBD rates declined. However, the decline in postneonatal mortality attributable to birth defects (PMBD) rate was significantly slower than that of overall postneonatal mortality. The adjusted rate ratio for non-Hispanic black and Hispanic versus non-Hispanic white for neonatal mortality attributable to birth defects (NMBD) remained unchanged from 1989–1991 through 2000–2002. For PMBD, it increased from 0.97 (95% confidence interval [CI], 0.90–1.13) in 1989–1991 to 1.12 (95% CI, 1.04–1.21) in 2001–2002 and from 1.08 (95% CI, 1.00–1.16) to 1.18 (95% CI, 1.10–1.27) for non-Hispanic black and Hispanic, respectively. Infant mortality due to cardiovascular and central nervous system defects were the main contributors to the increased racial disparities in PMBD rates. CONCLUSIONS: The disparity in PMBD between infants of non-Hispanic black and Hispanic mothers and infants of non-Hispanic white mothers increased significantly from 1989–1991 to 2000–2002. Further studies are needed to assess the extent to which delays in care or lack of access to care for infants with birth defects might be contributing to the disparity in IMBD. Birth Defects Research (Part A), 2006. © 2006 Wiley-Liss, Inc.