Neuronal ceroid lipofuscinosis (NCL; also known as Batten Disease) is a fatal neurodegenerative disorder that typically presents in childhood. Currently, no treatments are known that can halt or reverse the effects of NCL. A naturally occurring form of NCL analogous to late infantile-onset NCL in humans has been identified in a population of Japanese macaques (JMs). MRI examinations revealed marked cerebellar degeneration in NCL animals >4y/o (~14y/o equivalent human age) compared with controls, which is strikingly similar to human disease. This novel JM model presents a new opportunity for characterizing disease progression, identifying biomarkers, and pre-clinical therapeutic testing.