Neuronal ceroid lipofuscinsosis 1 (NCL1) is a rare lysosomal storage disease identified in a Miniature Dachshund. NCL1 is due to a deficiency in the Enzyme palmitoyl protein thioesterase (PPT1), which is necessary to break down certain proteins in the cells. As a result, there is an accumulation of these compounds in cells, which affects the normal function of the brain and nervous system. The affected dog presented with progressive neurologic disease at 9 months of age though the owner stated that the symptoms began a “number of months” earlier. Symptoms included a lack of muscle coordination, abnormal gait, and difficulty balancing and jumping. The dog also displayed signs of Dementia including aimless wandering behavior with episodes of confusion, depression, aggressive behavior, loss of learned behavior, blindness, seizures and frequent barking. These symptoms rapidly became more severe and the dog was euthanized at 14 months of age.