Cerebellar cortical atrophy has been described both in animals and in humans. In cattle, it has alreadybeen identified in several breeds, with the main histopathological lesions being located in the cerebellarPurkinje layer and ranging from chromatolysis to loss of neurons. This case, however, is the first described ina Belgian Blue cow. Additionally, the histopathological lesions were different from those described in othercattle breeds, with prominent focal granular layer loss and abnormal Purkinje cells comparable to thosedescribed in Norman-Jaeken disease or primary degeneration of the granular layer of the cerebellum inhumans.