A total of 438 brain samples from confirmed cases of transmissible spongiform encephalopathy (TSE) in sheep and goats in France collected in 1990 (216 samples), 2002 (135 samples) and 2003 (87 samples) were examined. The samples were screened using 3 western blot and 1 ELISA tests to detect the bovine spongiform encephalopathy (BSE) strain. The 4 tests provided convergent results from 1 goat sample (CH636), which had been identified out of 310 other goats at an abattoir during the 2002 survey; all the animals were destroyed. No prion protein was detected in the brain and lymphoid organs of the other goats. The same CH636 brain extract was intracerebrally inoculated into wild-type and transgenic mice strains. Incubation times were all compatible with those recorded for experimental ovine BSE. The presence of this strain in goats emphasizes the need to reassess the risks to human health linked to the consumption of small ruminant products.