Classification systems and case definitions provide the foundations upon which clinical and epidemiological studies are based. The European Research Network on Congenital Toxoplasmosis acknowledged the lack of such a system or definitions within its field of interest and established a working group to address the issue. Congenital Toxoplasma gondii infection was defined as occurring in four separate patient groups: pregnant women, fetuses, infants, and individuals > 1 year of age. The likelihood of Toxoplasma gondii infection was separated into five mutually exclusive categories: definite, probable, possible, unlikely, and not infected. Inclusion within a specific category is dependent upon the case definition, which is in turn derived from criteria based on serological, parasitological, and clinical information. Notes are included within the classification not only to clarify the definitions, but also to improve the reliability and quality of diagnosis. The goal is to construct a system that encompasses all aspects of congenital toxoplasmosis, which is applicable to different countries and health services, suitable for large epidemiological studies, aids the diagnosis and management of individual cases, and lends itself to computerisation.