Neuronal ceroid lipofuscinosis is an inherited, neurodegenerative lysosomal storage disease that affects humans, dogs, sheep, horses and cattle. Border Collies are affected in an autosomal recessive manner and serve as a model for CLN5, also known as Infantile neuronal ceroid lipofuscinosis (INCL) or Batten's disease, in people. Affected Border Collies are born neurologically normal, but begin to show signs by eighteen months of age and usually expire by thirty months. Definitive diagnosis can be made with genetic testing, but clinical signs, age of onset, MRI and retinal changes aid in heightening clinical suspicion. New therapies utilizing enzyme replacement, gene therapy, small molecular drugs and stem cell transplantation are in development, but have yet to be used in veterinary patients. Our patient was supportively treated and succumbed to her disease at two years of age. Necropsy and immunohistochemistiy were supportive of a diagnosis of this exceedingly rare disease.