Neuronal ceroidosis was observed in an 18-month-old male Blue Heeler dog which was euthanized after showing a progressive gait and behavior abnormality, depression, paresthesia, and vision deterioration. The brain was slightly atrophic. Histopathology revealed autofluorescent, periodic acid-Schiff, luxol fast blue, and oil red O-positive intracytoplasmic granules in the neurons of the brain and spinal cord. There was a moderate diffuse reduction in the number of cerebellar and cerebrocortical neurons. Ultrastructurally, these neuronal cytosomes were diverse with preponderance of membranous profiles and granular matrix.