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A neurodegenerative storage disease identified as ceroid-lipofuscinosis by light, fluorescence, and electron microscopic examinations was diagnosed in a 4-year-old female Cocker Spaniel dog with progressive ataxia and proprioceptive deficits. Stored pigment was found within neurons of the brain and spinal cord and in smooth muscle cells of the urinary bladder and small muscular arteries. The microscopic findings resembled those found in six other cases of generalized ceroid-lipofuscinosis in this breed. However, the brown discoloration of the intestines, which was the major gross lesion observed in those cases, was not found. This is the first report of the disease in Argentina.