In 2015, a young female Alpine chamois (
Rupicapra rupicapra rupicapra
), originated from the Aosta Valley Region, Northernwestern Italy, was conferred to the National Reference Centre for Wild Animal Diseases for pathologic examinations. Histological analysis revealed a severe meningoencephalitis characterized by lymphocytic and plasmacellular infiltration, gliosis, perivascular cuffs, and leptomeningitis at the level of brain and brain stem. Laboratory investigations included polymerase chain reaction, sequencing and characterization by phylogenetic analysis, and evaluation of the internal ribosome entry site secondary structure in the 5’ untranslated region. These tests identified the pathological agent as border disease virus, a known health risk in domestic small ruminants. Genetic characteristics of the isolated strains, closely related to ovine and caprine strain sequences from neighboring regions of Piedmont, France, and Switzerland, suggested geographic segregation and micro-evolutive steps within the species.