BACKGROUND: With the significant advancement of geographic information systems (GIS), mapping and evaluating the spatial distribution of health events has become easier. We examine the role of GIS in birth defects surveillance and research. METHODS: We briefly describe the geocoding process and potential problems in accuracy of the obtained geocodes, and some of the capabilities and limitations of GIS. We illustrate how GIS has been applied using the Metropolitan Atlanta Congenital Defects Program geocoded dataset. We provide some comments on potential data quality and confidentiality issues with birth defects in relation to GIS. RESULTS: It is desirable to geocode addresses using a multistrategy approach to achieve a high-quality and accurate GIS dataset. Beyond the basic but important function of mapping, sophisticated statistical approaches and software are available to analyze the spatial or spatial-temporal occurrence of birth defects, alone or in association with environmental hazards, and to present this information without compromising the confidentiality of the subjects. CONCLUSIONS: We recommend a broad and systematic use of GIS in birth defects spatial surveillance and research.